1

PEDIATRIC CARDIOLOGY
Congenital Heart Diseases:
Epidemiology
1. Incidence: In general incidence of CHD is 0.8%, and this incidence increases
to 26% for a 2nd birth;
2. When there are two 1st-degree relatives with CHD, the risk for a subsequent
child may reach 2030%
3. 34% in stillborn
4. 1025% in spontaneous abortuses
5. PDA in premature infants 2%

6. Mitral valve prolapse- 2-5 %

7. Bicuspid aortic valves - 12% in adults

LESION

% OF ALL
LESIONS

Ventricular septal defect

3530

Atrial septal defect (secundum)

68

Patent ductus arteriosus

68

Coarctation of aorta

57

Tetralogy of Fallot

57

Pulmonary valve stenosis

57

Aortic valve stenosis

47

d-Transposition of great arteries 35

Hypoplastic left ventricle

13

Hypoplastic right ventricle

13

Truncus arteriosus

12

Total anomalous pulmonary

venous return

12

Tricuspid atresia

12

Single ventricle

12

Double-outlet right ventricle

12

Others

510

Changes at birth
1. Ductus venosus closes over 3-10 days.
2. Fall in the pulmonary vascular resistance and increase in systemic resistance
3. Functional closure of the foramen ovale occurs within minutes to hours of
birth, anatomical closure takes several days
4. The ductus arteriosus constricts due to the high partial pressure of oxygen.
The process is usually complete within 2 days after birth.
Classification of CHD:
A. Acyanotic CHD
a) Left to right shunting:
i.
Intracardiac:
a. Septal: ASD, VSD,
b. Mixed: Endocardial Cushion Defect
c. Obstructive: AS, PS
d. Incompetent:
i. AV valves:
1. MVP; MI; TI;
ii. Semilunar valves:
1. Aortic Incompetence

ii.

iii.

2. Pulmonary Incompetence
Myocardium:
a. Cardiomyopathy
i. Obstructive
ii. Restrictive
iii. Dilated
Extracardiac:
a. PDA
b. AP window

B. Cyanotic:
Pulmonary
circulation
Increased
vascularity

Decreased
Vascularity

Ventricular
hypertrophy
RVH
LVH
RVH
RBBB
LVH
BVH

conditions
TGA; TAPVR; DORV; PPHN(NB)
Truncus arteriosus; single ventricle; TGA
with VSD
TOF; DORV with PS;
Epstein;
PS; tricuspid atresia
TGA with PS; Truncus arteriosus II & III;
single ventricle with PS

General features of Lt. to Rt. shunts:

1. Clinical signs are not always apparent at birth due to higher pressure in
pulmonary circulation.
2. They manifest anytime during infancy or early childhood as pulmonary
pressure reduced to normal levels.
3. Spontaneous closure possible with septal defects; unlikely in PDA
4. Increased pulmonary blood flow
5. Increased 02 saturation in the right side of the heart
6. Progressive Pulmonary hypertension
7. ventricular strain, dilation, and hypertrophy,
8. Recurrent respiratory infection
9. Feeding difficulty and failure to thrive
10.Mild defects manifest during severe exercise in adolescents and during
pregnancy
11.CCF
12.Eisenmenger complex a late complication

ATRIAL SEPTAL DEFECT

1. Epidemiology:
a. 7 % of CHDs;
b. 30 to 50 % as a part of all combined CHDs;
c. More in female (F:M=2:1)
2. Embryology:
1. Ostium primum : opening between septum primum and AV cushions ;
closed by the union of the septum primum by joining endocardiac cushion
2. Ostium secundum : opening in the upper part of the septum primum and
closed by septum secundum
3. Septum secundum : develops to the right of the septum primum
4. Foramen ovale: opening in the septum secundum; persists until birth and
closes as a depression called fossa ovalis.
5. Persistence of the ostium secundum is the most common atrial septal
defect.

3. Patent foramen ovale

a. Tunnel between septum primum and secundum
b. Functional closure at birth;
c. Probe patency for longer period in 25%
d. No intra cardiac shunts after birth
e. Adult stroke may be due to paradoxical embolus via PFO?
f. Migraine?
g. If increased RA pressure (pulmonary stenosis or atresia), R to L shunt
across the PFO may occur with resultant cyanosis.
h. In severe mitral stenosis, the foramen ovale may be sufficiently dilated
to result in left-to-right shunt.
4. ASD: Types:
1. Secundum: commonest; formed by failed growth of the septum secundum
; present at fossa ovalis; Anomalous pulmonary venous return in 10%
2. Primum: 30%; often part of endocardiac cushion defects;
3. Sinus venosus defect: 10%; between svc and RA
4. Coronary sinus defect: defect in the roof of coronary sinus connecting to
RA
5. Mitral valve prolapse occurs in 20%

4. Causes: The most common cause of an ostium primum ASD is genetic;

common in:
1. Trisomy 21.
2. Holt-Oram syndrome
3. Noonan syndrome
4. Ellis-van Creveld syndrome
5. Pathology
1. Dilated RA leads to RBBB in ECG
2. Mitral valve prolapse occurs in 20%

4
3. Partial anomalous pulmonary venous return, may be an associated lesion
4. Less commonly, the atrial septum may be nearly absent, with the creation
of a functional single atrium.
5. Heart block in occasional cases
6. Pathophysiology:
1. RV in early life is thick and less compliant limiting the L to R shunt
2. As the infant becomes older the left-to-right shunt increases as:
1. pulmonary vascular resistance drops,
2. the right ventricular wall becomes thinner
3. Left-to-right shunt is also determined by the size of the defect and the
relative compliance of the right ventricle (RV) and left ventricle (LV).
4. There is volume overload to RA, RV, PA and Pulmonary circulation. All
these chambers enlarge.
5. The increased blood flow results in enlargement of the right atrium and
ventricle and dilatation of the pulmonary artery.
6. The left atrium may also be enlarged, but the left ventricle and aorta are
normal in size.
7. Pulmonary vascular resistance remains low throughout childhood,
8. It may begin to increase in adulthood and may eventually result in
reversal of the shunt and clinical cyanosis
9. The dilated and stretched RV results in right bundle branch block (RBBB)
6. Clinical manifestations:
1. In NB and early infancy:
1. Ostium secundum ASD is most often asymptomatic; the lesion may
be discovered inadvertently during physical examination.
2. A relatively slender body build is typical.
2. In younger children:
1. subtle failure to thrive may be present;
2. Effort intolerance is indicated by suck-rest-suck cycle.
3. In older children:
1. Varying degrees of exercise intolerance may be noted.
4. Examination of the chest in a child with significant ASD:
1. Mild left precordial bulge.
2. A right ventricular parasternal systolic lift
3. A loud 1st heart sound and a pulmonic ejection click due to
increased flow through pulmonic valve
4. 2nd heart sound is widely split and fixed in its splitting in all
phases of respiration
5. Wide and fixed splitting of S2:
1. Wide splitting:
1. Lt. to Rt. shunt produce prolonged right ventricular
ejection and delayed pulmonary valve closure
2. RV dilatation produce RBBB resulting in delayed
closure of the pulmonary valve
2. Fixed splitting:
1. Fixed splitting indicates the presence of atrial
communication.
2. During inspiration more blood comes to RV from lungs
and during expiration more blood comes to RV through
ASD and RA and hence split of S2 remains fixed.
6. Murmurs:
1. No murmur at the ASD as pressure gradient is low

5
2. An ejection systolic murmur in pulmonary area; It is
produced by the increased flow across the pulmonary artery
3. A short, rumbling mid-diastolic murmur produced by the
increased volume of blood flow across the tricuspid valve is
often audible at the lower left sternal border; indicates a Qp :
Qs ratio > 2 : 1.
5. CCF:
1. In the first 3-4 years RV compliance is more and shunt is minimal
2. PA accommodates more blood by dilatation and hence no PH
initially
3. Hence, children with ASD rarely experience congestive heart
failure;
4. It can develop after PH becomes severe in the third and fourth
decades of life.
6. Investigations:
1. ECHO:
1. Increased right ventricular end-diastolic dimension
2. Flattening and abnormal motion of the ventricular septum
3. Brightening of the echo image seen at the edge of the defect (Tartefact).
4. Echo dropout can be seen in the midatrial septum
2. CXR:
1. RAH
2. RVH
3. Prominent PA
4. Increased pulmonary vascular markings
5. Normal LA
6. Jug Handle appearance of heart
3. ECG:
1. volume overload of the right ventricle;
2. the QRS axis may exhibit right axis deviation
3. A minor right ventricular conduction delay (rsR pattern in the
right precordial leads) may be present.
4. RBBB
4. Catheterization
1. Oxygen content of blood from the right atrium will be much
higher than that from the superior vena cava.
2. Pulmonary vascular resistance can be measured
10. Natural history:
1. Spontaneous closure in 40% in first 4 yrs.: > 8 cm closure is rare
2. Most children with an ASD remain active and asymptomatic.
3. CHF and pulmonary hypertension develop in adults who are in their
20s and 30s.
4. Atrial arrhythmias (flutter or fibrillation) may occur in adults.
5. Infective endocarditis does not occur in patients with isolated
ASDs.
6. Cerebrovascular accident, resulting from paradoxical embolization
through an ASD, is a rare complication
11. Treatment:
1. Medical:
1. Exercise restriction is unnecessary.
2. Prophylaxis for infective endocarditis is not indicated
3. In infants with CHF, medical management is recommended

12.

2. Indications for closure:

1. Any defect 8 mm or larger should be closed as soon as
diagnosed
2. After some years of follow-up, if the defect is 5 mm or larger, it
should be closed to prevent pulmonary vascular disease atrial
fibrillation
3. Closure:
1. Non-surgical:
a. Trans catheter device closure is advised for all
symptomatic patients and also for asymptomatic patients
with a Qp : Qs ratio of at least 2 : 1 or those with right
ventricular enlargement.
b. The timing for elective closure is usually after the 1st yr
and before entry into school.
c. Percutaneous catheter device closure using an atrial
septal occlusion device
d. Amplazer septal occluder by catheter
2. Surgical:
1. After 2-4 yrs. without significant PH
2. Repair is preferred during early childhood because surgical
mortality and morbidity are significantly greater in
adulthood; the long-term risk of arrhythmia is also greater
after ASD repair in adults
3. Teflon or pericardial patch closure
4. Mortality: It is less than 0.5%;
Prognosis;
1. Small- to moderate ASDs may close spontaneously.
2. Secundum ASDs are well tolerated until the 3rd decade or later.
3. Pulmonary hypertension, atrial dysrhythmias, tricuspid or mitral
insufficiency, and heart failure are late manifestations; these symptoms
may initially appear during the increased volume load of pregnancy.
4. Infective endocarditis is extremely rare, and antibiotic prophylaxis for
isolated secundum ASDs is not recommended.

VENTRICULAR SEPTAL DEFECT

1. VSD is the most common cardiac malformation and accounts for 25% of
congenital heart disease.15-20 % of CHDs;
2. Prevalence rate of 2.5 per 1000 live births; 1:1 M:F ratio
3. Embryology:
1. Failure of ventricular septation causes various forms of ventricular septal
defects (VSD).
2. If the ventricular ridge does not enfold, the result is a single ventricle
defect.
3. A lack of contribution of endocardial or conal tissue results in either a
high inlet, an outlet, or a membranous type VSD
4. The inlet: Septum from endocardial cushion tissue separates the mitral
and tricuspid valves. It is called "inlet" since it forms the area that allows
blood to enter the ventricles.
5. The outlet or infundibulam: Septum is formed by conal tissue. It is called
"outlet" because it forms the outflow part of the right ventricle, or
"infundibular" because it forms the area below the pulmonary artery.

4.

5.

6.

7.

6. The membranous septum: Is formed by the joining of the inlet and outlet
septum near the mitral and tricuspid valve. It is called "membranous"
because of the type of tissue it appears to be.
Associated defects:
1. VSDs between the crista supraventricularis and the papillary muscle of
the conus may be associated with pulmonary stenosis
2. VSDs superior to the crista supraventricularis (supracristal) are found just
beneath the pulmonary valve and may impinge on an aortic sinus and
cause aortic insufficiency.
Etiology:
1. In general incidence of CHD is 0.8%, and this incidence increases to 26%
for a 2nd birth
2. Two 1st-degree relatives with CHD, the risk for a subsequent child may
reach 2030%
3. Gene mutations result in ventricular septal defects eg. Holt-Oram
syndrome.
4. Chromosomal abnormalities (Trisomy)
5. May run in families;
6. Prevalence rate of 2.5 per 1000 live births; 1:1 M:F ratio
7. Part of other CHD: Eg. TOF
Location of defects: VSD may be classified as:
a) Membranous, (70%)
a. Perimembranous inlet (atrioventricular [AV] canal
type),
a. Perimembranous outlet
b) Muscular or Trabecular : 5% to 20%
a. Anterior
b. Midmuscular
c. Posterior
d. Apical
c) Swiss cheese type of multiple muscular defect
Pathophysiology:
1. Size of the VSD and pulmonary vascular resistance determine the shunt
magnitude
2. <5 mm the VSD is pressure restrictive meaning that right ventricular
pressure is normal. The higher pressure in the left ventricle drives the
shunt left to right.
3. >10 mm), right and left ventricular pressures are equalized. In these
defects, the direction of shunting and the shunt magnitude are
determined by the ratio of pulmonary to systemic vascular resistance
4. After birth in patients with a large VSD, pulmonary vascular resistance
may remain elevated and size of the left-to-right shunt may be limited.
5. pulmonary vascular resistance begins to fall in the 1st few weeks after
birth and the size of the left-to-right shunt increases.
6. When the ratio of pulmonary to systemic resistance approaches 1 : 1, the
shunt becomes bidirectional, signs of heart failure abate, and the patient
begins to show signs of cyanosis
7. In rare infants with a large VSD, usually those with Down syndrome,
pulmonary vascular resistance never decreases, and symptoms may
remain minimal until Eisenmenger physiology becomes evident.

8
8. The magnitude of intracardiac shunts is described by the Qp : Qs ratio. If
the left-to-right shunt is small Qp : Qs is <1.5 : 1. If the shunt is large Qp :
Qs is >2 : 1.

8. Hemodynamics:
1. Small VSD:
1. Pulmonary vascular congestion is minimal.
2. Minimal left ventricular hypertrophy
3. The shunt produces a loud, harsh, or blowing holosystolic murmur
and heard best over the lower left sternal border, and it is
frequently accompanied by a thrill.
4. A short, harsh systolic murmur localized to the apex in a neonate is
often a sign of a tiny VSD in the apical muscular septum. In
premature infants, the murmur may be heard early because
pulmonary vascular resistance decreases more rapidly
5. Intensity of the P2 is normal because the PA pressure is normal.
2. Moderate VSD:
1. There will be LVH
2. RVH is absent.
3. Pansystolic murmur is produced by the left-to-right shunt.
3. Large VSD:
1. LVH plus RVH and left atrial enlargement
2. Greatly increased pulmonary vascularity.
3. CHF in early infancy.
4. Eisenmenger's syndrome:
1. overall heart size decrease
2. Because of severe PH, there is persistence of RVH and prominent
PA
3. Because the shunt is small, the loudness of the murmur decreases,
4. The S 2 is loud and single owing to pulmonary hypertension
5. High altitude:
1. Hypoxia (high altitude) increases pulmonary vascular resistance
and decreases the amount of left-to-right shunt.
2. At high altitudes, children with ventricular defects develop
congestive heart failure less commonly than at sea level
9. Clinical:
Infancy:
1. Small defect:
a)
Most infants are asymptomatic because most defects are
too small to allow sufficient left-to-right shunting to cause
symptoms.

b)

Murmurs are rarely audible at birth owing to pulmonary

hypertension(normal)
2. Larger defects:
a)
May produce tachypnea, symptoms of congestive failure,
dyspnea, reduced feeding (suck-rest-suck cycle), and failure to
thrive.
b) Respiratory infections are frequent (respiratory syncytial virus
is common)
Older children:
1. Small or moderate-sized defects:
c)
Pansystolic loud murmur with a thrill best heard at the
third and fourth left intercostal spaces at the sternal border;
d)
The very loud systolic murmur in small membranous
defect (maladie de Roger syndrome)
2. In large defects:
a. Infant with a large defect is malnourished and scrawny(bony)
b. Feeding difficulty with profuse perspiration; suck-rest cycles
c. Respiratory rate is 80 to 100 breaths/minute
d. The liver edge is palpable well below the right costal margin
e. The cardiac impulse is hyperdynamic. Prominent left precordium is
common
f. Because there is no pressure gradient across the ventricular defect,
the systolic murmur is not loud
g. Diastolic flow rumble due to excessive mitral valve flow is present;
This indicates Qp : Qs ratio of 2 : 1
h. Peripheral pulses are good (small volume collapsing)
i. Second heart sound is narrowly split and the pulmonary component
of is accentuated.
j. Wheeze due to pressure by the enlarged left atrium on the bronchus
2. Eisenmenger complex:
1. End result of Rt to Lt shunts
2. In this complex there is severe Pulmonary hypertension
3. RVH more; LVH is less prominent
4.
The pulmonary component of the second heart sound is
very loud,
5.
Pul. Incompetent murmur is audible.
6. The pan systolic murmur disappears
7. The patient is mildly cyanosed.
8. Pt is Inoperable.
10.ECG:
1. With a small VSD, the ECG is normal.
2. With a moderate VSD, left ventricular hypertrophy (LVH) and occasional
left atrial hypertrophy (LAH) may be seen.
3. With a large defect, the ECG shows biventricular hypertrophy (BVH) with
or without LAH
4. P waves may be notched or peaked.
11. CXR:
1. Cardiomegaly of varying degrees is present and involves the LA, left
ventricle (LV), and sometimes RV.
2. Pulmonary vascular markings increase.

10
3. In Eisenmenger's complex, the main PA and the hilar PAs enlarge
noticeably, but the peripheral lung fields are ischemic. The heart size is
usually normal
12.Echo:
1. Selected two-dimensional echo views of the ventricular septum and the
site of a VSD.
2. Color-flow Doppler mapping identifies color-flow jets across the ventricular
defect
3. The echocardiogram can also be useful to determine the presence of
aortic valve insufficiency or aortic leaflet prolapse in the case of
supracristal VSDs
13.
Cardiac catheterization:
1. Oximetry demonstrates increased oxygen content in the right ventricle
2. Small, restrictive VSDs are associated with normal right heart pressures
3. The size, location, and number of ventricular defects can be
demonstrated by left ventriculography. Contrast medium passes across
the defect to opacify the right ventricle and pulmonary artery.
14.
Natural History:
a. Spontaneous closure:
i. A significant number (30-50%) of small defects close
spontaneously, most frequently in < 2 yrs. of life & majority before
4 yrs.
ii. Small muscular VSDs are more likely to close (up to 80%) than
membranous VSDs
iii. Outlet and Infundibular defects do not close
b. Child with small defect:
i. Most children with small defects remain asymptomatic,
ii. A long-term risk is infective endocarditis.
c. Child with moderate to large VSD:
i. May become smaller and up to 8% may close completely.
ii. More commonly, repeated episodes of respiratory infection and
heart failure
iii. Failure to thrive.
iv. Pulmonary hypertension
d. Child with supracristal VSD:
i. Patients with VSD are also at risk for the development of aortic
valve regurgitation, the greatest risk occurring in patients with
supracristal VSD.
e. VSD with infundibular stenosis (Acyanotic Fallot): some may develop
acquired infundibular stenosis; the clinical picture changes to a VSD with
pulmonary stenosis.
15.
Treatment:
Medical:
1. CHF: digoxin and diuretics; captopril reduces after load; spironolactone to
minimize potassium loss
2. Oral iron to correct anemia
3. Diet:
a) Frequent feedings of high calorie diet;

11

b)

Expressed breast milk or formula contains 20

calories/ounce; can be supplemented with carbohydrate or fat to
provide 30 calories/ounce
c)
Corn oil can be added to EBM or formula milk given as
extra milk after breast feeding
4. Exercise as per tolerance if there is no PH
5. Dental hygiene and AB prophylaxis during extraction
Closure:
Non-surgical: umbrella device closure through catheter
Surgical:
1. Indications for surgical closure:
1. Subpulmonary defects soon after diagnosis
2. PA pressure greater than 50% of systemic pressure,
3. significant left-to-right shunt with Qp/Qs ratio > 2:1
4. Aortic valve prolapse into outlet VSD
5. Single episode of endocarditis
2. Procedure: trans atrial or trans PA or ventriculotomy and patching
with synthetic or pericardial tissue.

PDA
Embryology:
1. The ductus arteriosus is derived from the left sixth embryologic arch
2. It connects the origin of the left main pulmonary artery to the aorta, just
below the left subclavian artery
3. In patients with tetralogy of Fallot, the ductus is often absent.
4. PDA is functionally closed in 48 hours and anatomically closed in 10 days due
to increased O2 concentration and release of bradykinin
5. It takes longer periods in premature infants
6. The ductus is becomes the ligamentum arteriosum.
Incidence:
1. 0.8 per 1000 live births
2. 5-10% of CHDs;
3. More in female with 2:1 ratio
4. common in preterm
Causes of PDA:
1. Premature birth as the smooth muscle in the wall of the preterm ductus is
less responsive to high Po2 and therefore less likely to constrict after birth.
2. PDA in a term infant rarely closes spontaneously, whereas in a premature
infant, spontaneous closure occurs in most instances.
3. A PDA is seen in 10% of patients with other congenital heart lesions
4. perinatal hypoxia
5. children born at extreme altitude
6. Familial; 5% among siblings
7. Congenital Rubella syndrome
8. Fetal alcohol syndrome,
9. Maternal amphetamine and phenytoin use.
Hemodynamics:

12
1. similar to VSD
2. Duct is post Lt. subclavian connecting descending aorta and Lt. PA
3. large runoff from the aorta to the pulmonary artery and excessive blood flow
to the lungs, left atrium, left ventricle, and ascending aorta and enlargement
of these structures is in proportion to the size of the left-to-right shunt
4. shunt occurs both in diastole and systole producing continuous murmur
Pathophysiology:
1. In small shunt no PH and P2 is normal
2. In large shunt PH develops leading to RVH and accentuated P2 with diastolic
murmur in mitral area due excessive mitral valve flow.
3. As PH progresses bidirectional develops with differential cyanosis: upper
limbs pink and lower limbs blue
4. Further progress of PH leads to Eisenmenger syndrome
Clinical:
1. Infants up to 8 weeks: no symptoms as high pulmonary vascular resistance
prevents L to R shunt
2. No symptoms in small ductus
3. Large ductus leads to:
1. Poor weight gain
2. Recurrent respiratory infections
3. CHF
4. On examination:
1) Tachycardia
2) Tachypnea
3) Exertional dyspnoea
4) Bounding and collapsing pulse as blood runs off from the aorta into the
pulmonary circulation.
5) Wide pulse pressure
6) Hyperactive precardium; the apical impulse is laterally displaced.
7) A thrill may be present in the suprasternal notch or in the left
infraclavicular region.
8) The first heart sound (S1) is normal. The second heart sound (S 2) is
obscured by the murmur.
9) Paradoxical splitting of S2 related to premature closure of the
pulmonary valve
10)
Gibsons machinery murmur:
a. This is continuous & loudest located in the second left
intercostal space
b. In the small infant, it is uncommon to hear the diastolic
component of the murmur
c. It may radiate down the left sternal border or to the left clavicle.
d. When pulmonary vascular resistance is increased, the diastolic
component of the murmur may be less prominent or absent.
11)
Apical diastolic rumble, caused by high flow through mitral valve
into the left ventricle is audible in large PDA
ECG:
a. If the left-to-right shunt is small, the electrocardiogram is normal;
b. If the ductus is large, left ventricular or biventricular hypertrophy is
present.
CXR:
1. Large PDA shows a prominent pulmonary artery with increased pulmonary
vascular markings.

13
2. Moderately to markedly enlarged chambers are the left atrium and left
ventricle.
3. The aortic knob may be normal or prominent.
4. In Eisenmenger complex: reduced heart size; increased PA segment;
prominent hilar vessels
ECHO:
1. With large shunts, left atrial and left ventricular dimensions are increased.
2. The ductus can easily be visualized directly and its size estimated.
3. Color and pulsed Doppler examinations demonstrate systolic retrograde
turbulent flow in the pulmonary artery, and aortic retrograde flow in
diastole
Cardiac catheterization:
1. Cath. will demonstrate either normal or increased pressure in the right
ventricle and pulmonary artery, depending on the size of the ductus.
2. The presence of oxygenated blood shunting into the pulmonary artery
confirms the left-to right shunt.
3. The catheter may pass from the pulmonary artery through the ductus into
the descending aorta. Injection of contrast medium into the ascending
aorta shows opacification of the pulmonary artery from the aorta and
identifies the ductus.
Natural history:
a. No spontaneous closure of PDA is possible in term infants
b. Recurrent pneumonia
c. CHF
d. Infective endocarditis
e. Aneurismal dilatation of PDA and rupture
f. Eisenmenger complex
DD: of continuous murmur
No Lesion
Differentiating points
1
2

Coronary AV fistula
Systemic AVF

Pulmonary AVF

Venous hum

5
6

VSD with AR
persistent truncus
arteriosus
Pulmonary artery
stenosis

7
8
9

Total anomalous pul.

venous return
ruptured sinus of
Valsalva

Murmur at Rt. sternal border

Bounding pulse but no murmur over
precardium
Murmur over back with cyanosis and
clubbing
Rt. infra and supra clavicular;
disappears in supine position
Murmur in lower left sternum
Cyanosis; BVH; murmur in Rt
intercostal spaces
Continuous murmur all over chest;
RVH; in Rubella and Williams syndrome
Venous hum over Rt. sternal border;
cyanosis; RVH
not previously heard, which most often
is also loudest at the second left
interspace.

Prognosis and complications:

1. Spontaneous closure of the ductus after infancy is extremely rare.

14
2. Patients with a small PDA may live a normal span with few or no cardiac
symptoms, but late manifestations may occur.
3. In patients with a large PDA, cardiac failure most often occurs in early infancy
4. Infective endarteritis may be seen at any age.
5. Pulmonary or systemic emboli may occur.
6. Rare complications include aneurysmal dilatation of the pulmonary artery or
the ductus, calcification of the ductus, noninfective thrombosis of the ductus
with embolization, and paradoxical emboli.
7. Pulmonary hypertension (Eisenmenger syndrome) usually develops in
patients with a large PDA who do not undergo ductal closure
Management:
a. Irrespective of age, patients with PDA require surgical or catheter closure.
b. Closure of the ductus is indicated in symptomatic patients, preferably
before 1 yr of age.
c. Closure is essential for
i. Prevention of bacterial endarteritis
ii. Heart failure
iii. Prevent the development of pulmonary vascular disease
d. Small PDAs are closed with intravascular coils.
e. Moderate to large PDAs may be closed with an umbrella-like device or
with a catheter introduced sac into which several coils are released.
f. Surgical closure of PDA can be accomplished by a standard left
thoracotomy or using thoracoscopic minimally invasive techniques.
i. Ligation and division through left posterolateral thoracotomy
without cardiopulmonary bypass
ii. video-assisted thoracoscopic clip ligation
g. Complications of surgery
i. Complications are rare. Mortality is < 1%.
ii. Injury to the recurrent laryngeal nerve (hoarseness), the left
phrenic nerve (paralysis of the left hemidiaphragm), or the thoracic
duct (chylothorax) is possible.
iii. Recanalization (reopening) of the ductus is possible, although rare,
occurring after ligation alone (without division).
h. Pulmonary hypertension is not a contraindication to surgery at any age if
the shunt flow is still predominantly left to right and that severe
pulmonary vascular disease is not present.
i. Indomethicin for preterm (not for term babies) : 0.2 mg IV infusion 12 hrly
3 doses; or ibuprofen 10 mg/kg iv for 3 days;
j. Others:
i. SBE prophylaxis
ii. CCF management
Eisenmenger syndrome
1. The term Eisenmenger syndrome refers to patients with a ventricular septal
defect in which blood is shunted partially or totally from right to left as a
result of the development of pulmonary vascular disease. This physiologic
abnormality can also occur with atrioventricular septal defect, ventricular
septal defect, patent ductus arteriosus or any other communication between
the aorta and pulmonary artery, and in many forms of complex congenital
heart disease with unrestricted pulmonary blood flow.

15
2.

3.

4.
5.

6.

7.

The pathologic changes of Eisenmenger syndrome are graded on the basis

of histologic characteristics (Heath-Edwards classification):
a. Grade I changes involve medial hypertrophy alone,
b. grade II consists of medial hypertrophy and intimal hyperplasia,
c. grade III involves near obliteration of the vessel lumen,
d. grade IV includes arterial dilatation, and grades V and VI include
plexiform lesions, angiomatoid formation, and fibrinoid necrosis.
e. Grades IV-VI indicate irreversible pulmonary vascular obstructive
disease.
Eisenmenger physiology is defined by an absolute elevation in pulmonary
arterial resistance to greater than 12 Wood units (resistance units indexed to
body surface area) or by a ratio of pulmonary to systemic vascular resistance
of 1
Pulmonary vascular disease occurs more rapidly in patients with trisomy 21
who have left-to-right shunts.
CLINICAL MANIFESTATIONS:
a. Symptoms develop until the 2nd or 3rd decade of life
b. Cyanosis becomes apparent, and dyspnea, fatigue, and a tendency
toward dysrhythmias begin to occur.
c. In the late stages of the disease, heart failure, chest pain, headaches,
syncope, and hemoptysis may be seen.
Physical examination;
a. It reveals a right ventricular heave and a narrowly split 2nd heart
sound with a loud pulmonic component.
b. Palpable pulmonary artery pulsation may be present at the left upper
sternal border.
c. A holosystolic murmur of tricuspid regurgitation may be audible along
the left sternal border.
d. An early decrescendo diastolic murmur of pulmonary insufficiency may
also be heard along the left sternal border.
e. The degree of cyanosis depends on the stage of the disease.
DIAGNOSIS
Roentgenogram:
a. The heart varies in size from normal to greatly enlarged
b. The main pulmonary artery is generally prominent. The pulmonary
vessels are enlarged in the hilar areas and taper rapidly in caliber in
the peripheral branches.
c. The right ventricle and atrium are prominent. The electrocardiogram
shows marked right ventricular hypertrophy. The P wave may be tall
and spiked.
d. Cyanotic patients have various degrees of polycythemia that depend
on the severity and duration of hypoxemia.
The echocardiogram:
a. Shows a thick-walled right ventricle and demonstrates the underlying
congenital heart lesion.
b. Twodimensional echocardiography assists in eliminating from
consideration lesions such as obstructed pulmonary veins, supramitral
membrane, mitral stenosis, and restrictive cardiomyopathy.
c. Doppler studies demonstrate the direction of the intracardiac shunt
and the presence of a typical hypertension waveform in the main
pulmonary artery.
Cardiac catheterization:

16
a. Shows a bidirectional shunt at the site of the defect.
b. Systolic pressure is generally equal in the systemic and pulmonary
circulations.
c. Pulmonary capillary wedge pressure is normal
d. Arterial oxygen saturation is decreased depending on the magnitude
of the right-to-left shunt.
8. Management:
a. Prevention is by early surgical elimination of large intracardiac or great
vessel communications during infancy.
b. Delayed recognition is a particular risk in patients with congenital
heart disease who live at high altitude. It is also a risk in infants with
trisomy 21, who have a propensity for earlier development of
pulmonary vascular disease. Because of the high incidence of
congenital heart disease associated with trisomy 21, routine
echocardiography is recommended at the time of initial diagnosis,
even in the absence of other clinical findings.
c. Medical treatment:
i. Oral: calcium channel blocker, endothelin antagonist,
phosphodiesterase inhibitors
ii. Chronic intravenous prostacyclin therapy.
d. Combined heart-lung or bilateral lung transplantation is the only
surgical option for many of these patients
Pulmonary valvular Stenosis (PS):
1. 8-12% of CHDs
2. Associated with TOF and single ventricle
3. Types:
a. Valvular ( Noonan syndrome)
b. Sub valvluar
c. Supravalvular ( Rubella, Williams, Ehrles Danlos, Silver Russel syndromes)
4. Clinical:
a. Mild: no symptoms
b. Severe: dyspnoea on exertion; easy fatigability
c. Newborn: cyanosis ; poor feeding
d. Signs:
i. Apical tap
ii. Systolic thrill in Lt. upper sternal border and suprasternal notch
iii. Systolic ejection click in pulmonary area
iv. Soft P2 and wide split
v. Ejection systolic murmur in Lt. upper sternal border conducted to
back; longer the murmur severe the stenosis
vi. Hepatomegaly
5. Peripheral pulmonary branch stenosis: Mid systolic murmur in pulmonary area
conducted to axilla and back; sometimes continuous murmur.
6. ECG: RAD; RVH; LVH in NB
7. CXR:
a. Heart size normal or increased;
b. PA prominent with post stenotic dilatation
c. Decreased pulmonary vascular markings
8. Echo: thick valve cusps; restricted motion; PA dilatation.
9. Natural history:
a. AS is not progressive
b. CHF in severe stenosis

17
c. Infective endocarditis occasional
d. Sudden death during strenuous exercise
e. PS in NB has high mortality
10.Treatment:
a. NB:
i. PGE1 infusion to reopen ductus arteriosus
ii. Emergency balloon valvuloplasty during catheterization
b. Balloon angioplasty with stainless steel stent for supra valvluar stenosis
c. Antibiotic prophylaxis for SBE
d. Surgery:
i. Transventricular valvotomy
ii. Patch widening for Infundibular stenosis
iii. Systemic PA shunting
Aortic Stenosis:
1.
2.
3.
4.
5.

10 % of CHDs
Types: Infra valvluar; valvular( most common); supra valvular
Valvular : bicuspid, unicuspid or stenosis of tricuspid valve
Critical stenosis in NB: pin hole opening; hypoplasia of aorta and LV
Pathology:
a. Stenosis due to fusion of leaflets and small orifice
b. Annular constriction of upper margin of sinus of Valsalva is the pathology
in supravalvular stenosis
c. Infra valvular stenosis: eg. Idiopathic hypertrophic sub aortic stenosis that
occurs in one type of Cardiomyopathy.
6. Clinical:
a. NB: may develop pulmonary oedema and respiratory distress
b. Exercise intolerance
c. Exertional chest pain
d. Syncope
7. Physical findings:
a. Acyanotic
b. Narrow pulse wave
c. Systolic thrill in Rt. upper sternal border, supra sternal notch and over
carotid arteries
d. Ejection click in aortic area
e. Paradoxical splitting of S2 in pulmonic area
f. Mid systolic murmur in II Rt. intercostal conducted to neck and apex.
g. Early diastolic murmur due to AI
h. NB: week pulse, pallor, cold extremities which may mimic sepsis.
8. Signs of Severity:
1. Single S2 or paradoxical splitting
2. Longer murmur
3. Late peaking of murmur.
9. EEG; LVH
10.CXR:
a. Prominent aorta and knuckle due to post stenotic dilatation
b. Cardiomegaly due to CCF
c. NB: Cardiomegaly with plethoric lungs
11.Echo: heavy Y pattern valve cusps; small orifice
12.Natural history:
a. Syncope
b. Sudden death
c. CCF

18
d. Stenosis progress with age
e. Aortic incompetence can occur
f. SBE in 4 %
13.Management:
a. Medical:
i. NB: PGE1 infusion to open PDA
ii. Balloon valvuloplasty
b. Monitor progression of AS
c. Oral hygiene and SBE prophylaxis
d. Restriction of strenuous exertion in severe AS
e. Surgery:
i. Valvular:
1. Balloon valvulotomy
2. Commissurotomy
3. Valve replacement: Ross procedure: pulmonary valve fitted
to aortic and pulmonary valve reconstructed by allograft.
ii. Sub valvular: Removal of membrane
iii. Supra valvular: Y patch reconstructive surgery
Coarctation of Aorta:
1. Constrictions of the aorta of varying degrees may occur at any point from the
transverse arch to the iliac bifurcation, but 98% occur just below the origin of
the left subclavian artery at the
origin of the ductus arteriosus (juxtaductal coarctation).
2. The anomaly occurs twice as often in males as in females. Coarctation of the
aorta may be a feature of Turner syndrome
3. It is associated with a bicuspid aortic valve in more than 70% of patients.
4. Mitral valve abnormalities (a supravalvular mitral ring or parachute mitral valve)
and subaortic stenosis are potential associated lesions. When this group of leftsided obstructive lesions occurs together, they are referred to as the Shone
complex.
5. It is postulated that coarctation may be initiated in fetal life by the presence of a
cardiac abnormality that results in decreased blood flow anterograde through
the aortic valve (e.g., bicuspid aortic valve, VSD). Alternatively, coarctation may
be due to abnormal extension of contractile ductal tissue into the aortic wall.
1. Incidence: 0.2/1000 live births ; 8-10% of total CHDs; more in male; 30% in
Turner
2. Pathology:
1. Usually juxta ductal
2. Collaterals:
1. Intercostal from subclavian
2. Internal memory
3. Ant. Spinal artery
3. Bicuspid aortic valve in 85%
4. Spectrum: Severe that manifests in NB or asymptomatic type
3. NB and infants:
1. Poor feeding; dyspnoea and poor wt gain
2. Shock
3. Shunt:
1.
Left-to-right ductal shunting leading to pulmonary hypertension.
2.
In severe coarctation, Rt. to Lt. shunt and venous blood from RV
pass through ductus to lower extremities produces differential

19

4.

5.
6.

7.

8.

cyanosis with the upper extremities being pink and the lower
extremities blue
4. S2 single and loud; S3 gallop; no murmur
5. CHF in 3 months and death
Childhood:
1. May grow up normally if coarctation is less severe.
2. Some children or adolescents complain about weakness or pain (or both)
in the legs
3. Older children are frequently found to be hypertensive on routine physical
examination
4. BP in upper limbs more or equal to lower limbs (normally 20 mm Hg more
in LL)
5. A pressure higher in the right than the left arm suggests involvement of
the left subclavian artery in the area of coarctation.
6. With exercise, a more prominent rise in systemic blood pressure occurs
7. Diastolic hypertension in the lower extremities, despite a systolic pressure
gradient, suggests hypertension of some other cause (i.e., abdominal
coarctation with involvement of the renal arteries).
8. The femoral, popliteal, posterior tibial, and dorsalis pedis pulses are weak
(or absent in up to 40% of patients)
9. Bounding pulses of the arms and carotid vessels.
10.When the radial and femoral pulses are palpated simultaneously, a radialfemoral delay could be made out.
11.S2 split normally A2 accentuated
12.A systolic ejection click or thrill in the suprasternal notch suggests a
bicuspid aortic valve
13.A short systolic murmur is often heard along the left sternal border at the
3rd and 4th intercostal spaces. The murmur is well transmitted to the left
infrascapular area and occasionally to the neck.
14.In older patients with well-developed collateral blood flow, systolic or
continuous murmurs may be heard over the left and right sides of the
chest laterally and posteriorly. In these patients, a palpable thrill can
occasionally be appreciated in the intercostal spaces on the back
15.Diastolic murmur due to aortic regurgitation
16.Ejection systolic murmur in Rt. Upper sternal border
ECG: LVH
CXR:
1. Cardiac enlargement and pulmonary congestion
2. The enlarged left subclavian artery produces a prominent shadow in the
left superior mediastinum.
3. In most instances, the descending aorta has an area of poststenotic
dilatation.
4. E filling defect in barium swallow
5. 3 sign of aortic shadow in over penetrated picture
6. Rib notching in 4-8 ribs in child >5 yrs
Echo:
1. Left ventricular hypertrophy in older patients. Neonates and young infants
display right or biventricular hypertrophy.
2. suprasternal echo shows shelf like membrane in descending aorta and
post stenotic dilatation
3. Coarctation can be visualized by two-dimensional echocardiography
Natural History:
1. Bicuspid AV valve may cause stenosis and regurgitation

20
2. SBE
3. LVF
4. Rupture of aorta
5. Intra cranial haemorrhage
6. Hypertensive encephalopathy
7. Hypertensive CCF
9. Treatment:
1. NB should be given an infusion of prostaglandin E1 to reopen the ductus
and re-establish adequate lower extremity blood flow.
2. Anticongestive measures to improve clinical status before surgical
intervention.
3. Dental hygiene and SBE prophylaxis
4. There is usually no reason to delay surgical repair waiting for patient
growth; successful repairs have been performed in small premature
infants.
5. Older children with significant coarctation of the aorta should be treated
relatively soon after diagnosis
6. Non surgical:
1. Balloon angioplasty
2. Stainless steel stent
7. Surgical:
1. Dissection and end to end anastomosis
2. Subclavian artery aortoplasty
8. Bicuspid valve needs lifelong follow up and surgery is not total cure
10.POSTCOARCTECTOMY SYNDROME
1. In the immediate postoperative course, rebound hypertension is
common and requires medical management.
2. Postoperative mesenteric arteritis may be associated with acute
hypertension and abdominal pain in the immediate postoperative period.
11.PROGNOSIS
1. Although re-stenosis in older patients after coarctectomy is rare, a
significant number of infants operated on before 1 yr of age require
revision later in childhood
2. Subarachnoid or intracerebral hemorrhage may result from rupture of
congenital aneurysms in the circle of Willis
3. Aneurysms of the descending aorta or the enlarged collateral vessels may
develop.
Endocardial Cushion Defect:
1. Basics:
1. Endocardial cushion defects, more commonly known as atrioventricular
(AV) canal or septal defects include a range of defects characterized by
involvement of the atrial septum, the ventricular septum, and one or both
of the AV valves.
2. A complete AV septal defect indicates the presence of both atrial and
ventricular septal defects with a common AV valve (see image below). A
partial defect indicates atrial septal involvement with separate mitral and
tricuspid valve orifices.
3. AV canal defects arise from abnormal development of the endocardial
cushions. The superior and inferior cushions do not close completely. An
interatrial communication is left at the lower portion of the atrial septum.
This is called an ostium primum defect. The failure of the endocardial

21

2.
3.

4.

5.
6.
7.
8.

cushions to fuse results in an abnormally low position of the AV valves and

an abnormally high position of the aortic valve. A portion of the AV valves
originates from the endocardial cushions and their improper fusion results
in anterior and posterior components to the mitral valve leaflet.
Incidence: 2 % of CHDs; 70 % of cases are Down syndrome and 40 % Down
will have ECD;
Pathology:
1. ASD
2. VSD
3. Common AV valve
4. 2 AV valve in partial defect
5. Lt. to Rt. shunt
Clinical:
1. S1 accentuated; S2 narrow split
2. Pansystolic murmur Lt. sternal border due to mitral regurgitation
3. Mid diastolic rumble due to relative mitral/tricuspid stenosis
CXR: 4 chamber Cardiomegaly; PH
ECG: Prolonged P-R; RBBB
Echo: subcostal 5 chamber view demonstrates goose neck deformity
Surgery: early surgery before PH develops.
CYANOTIC HEART DISEASES
1. Causes of cyanosis:
1. CVS:
not improved by O2 and aggravated by crying
2. RS:
}
improve while crying and by O2
3. CNS depression
}
2. Classification:
Pulmonar
y
circulation
Increased
vascularity
Decreased
Vascularity

Ventricula
r
hypertrop
hy
RVH
LVH
RVH
RBBB
LVH
BVH

conditions

TGA; TAPVR; DORV; PPHN(NB)

Truncus arteriosus; single ventricle; TGA with VSD
TOF; DORV with PS;
Epstein;
PS; tricuspid atresia
TGA with PS; Truncus arteriosus II & III; single
ventricle with PS

TRANSPOSITION OF GREAT ARTERIES:

1. Incidence: 5-7%; female: male 3:1
2. Pathology:
1. Aorta arises anteriorly and to Rt. of PA from RV (D-transposition)
2. PA arises posteriorly from LV
3. 50% have PFO or PDA
4. 5% have PS
5. 30-40% have VSD
6. 10% have VSD+PS
3. Clinical:
1. Common in large for date NB
2. Cyanosis and CCF soon after birth
3. S2 single and loud

22

4.
5.
6.
7.

8.

4. No murmur
5. Pan systolic if VSD is present
6. Soft systolic murmur in PS is present
7. Hepatomegaly if CCF is present
8. Hypoglycaemia and hypocalcaemia can occur
ECG: Rt axis deviation; RVH; BVH if VSD or PDA is present;
CXR: Cardiomegaly with pul.plethora; Egg shaped heart
ECHO:
1. Parallel alignment of great arteries
2. Double circle in parasternal short axis echo
Natural history:
1. Death in NB or before 6 mo
2. Intact ventricular septum is a severe form
3. VSD reduces cyanosis but increases PH
4. PS with VSD longer survival but more surgical risk; less PH
Management:
1. Medical: Emergency measures:
1. PGE1 infusion to open PDA
2. O2 to reduce PH
3. Balloon atrial septostomy (Raskind procedure)
4. Digoxin and diuretics
2. Surgical:
i. Atrial level: (Semiz or Mustard) pul. and systemic circulation
rerouted at atrial level
ii. Ventricular level: (Rastelli) tunnel between VSD and aortic valve
iii. Arterial switch: proximal portions of great arteries connected to
distal portions after switching; coronary artery transplanted to PA
TETROLOGY OF FALLOT

1. Incidence:
a. 5-10% of CHDs;
b. In siblings of affected parents it is 1-5%
c. More common in males than in females;
d. 3 per 10,000 live births ;
e. The risk of recurrence in families is 3%.
2. The aetiology is multifactorial, but reported associations include:
a. Maternal:
i. untreated maternal diabetes,
ii. Phenylketonuria,
iii. Intake of retinoic acid.
b. Fetal alcohol syndrome
c. Maternal use of hydantoin and carbamazepine
d. Fetal:
i. DiGeorge syndrome
ii. Down syndrome
iii. Associated chromosomal anomalies:
1. Trisomies 21, 18, and 13,
2. Microdeletions of chromosome 22 is more common
iv. Gene mutations:
1. TBX1 gene,
2. JAG1 in Alagille syndrome.
3. TBX5 in Holt-Oram syndrome,
3. The basic pathology of Tetralogy:

23

4.

5.

6.

7.

a. Underdevelopment of the right ventricular infundibulum (malalignment

of the conal septum), which results in an anterior-leftward
malalignment of the Infundibular septum.
Pathology of components:
1. Large VSD:
i. Nonrestrictive and large; perimembranous defect; equalizes
pressures in both ventricles
ii. The aortic root is large and overrides the VSD
2. RVOT obstruction:
i. Common type is infundibular stenosis (45%).
ii. The obstruction is rarely at the pulmonary valve level (10%).
3. RVH: due to infundibular stenosis and systolic pressure which is equal
to left ventricle
4. Over riding of aorta:
i. The overriding aortic root is enlarged in tetralogy of Fallot;
ii. Rt sided aortic arch in 25%
iii. Abnormal coronary artery in 5%
5. Occasional ASD (Pentology)
Subgroups:
1. Tetralogy with pulmonary atresia,
2. TOF with absent pulmonary valve,
3. TOF with common atrioventricular canal
Physiology:
1. Cyanosis is directly proportional to right ventricular outflow
obstruction.
2. If RVOT obstruction is mild, child may not have cyanosis - pink
tetralogy.
3. CCF is uncommon as both ventricles can easily eject blood into
overriding large aorta.
4. If VSD is large and pulmonary obstruction is less increased Lt to Rt
shunt can lead to CCF.
5. Pulmonary Infundibulum is muscular and can undergo spasm (Dynamic
muscular constriction) leading to cyanotic spells;
6. Muscular infundibulum grows with age with increasing stenosis and
cyanosis
Clinical:
1. The infant initially manifest a murmur, with or without cyanosis
2. The murmur is systolic ejection murmur due to infundibular stenosis;
well heard along the left sternal border
3. The second heart sound is single due to aortic component
4. There is a right ventricular parasternal lift.
5. Cyanosis:
1. Infants at birth may be entirely asymptomatic
2. Later, dyspnea on exertion, squatting, or hypoxic spells develop
3. Severe cyanosis is seen in patients with TOF and pulmonary
atresia
6. Hyper Cyanotic spell:
1. Hypoxic spells occur with a peak incidence between 2 and 4
months of age.
2. Occurs in Tetralogy of Fallot and pulmonary atresia.

24
3. Paroxysmal; the onset is usually spontaneous and unpredictable.
4. The spells occur most frequently in the morning or after episodes
of vigorous crying or during stress or dehydration ie. periods of
increased oxygen demand.
5. The infant becomes hyperpneic and restless, cyanosis increases,
gasping respirations ensue.
6.

May progress to unconsciousness and, occasionally, to convulsions

or hemiparesis and even death.

7. Temporary disappearance systolic murmur as flow across the right

ventricular outflow tract diminishes.
8. Spell may be self limiting of spells are self-aggravating with
severe systemic hypoxia and metabolic acidosis.
9. The pathophysiology is not fully understood, but relates to:
1.

Increase in Infundibular spasm due to catecholamines

release in stress and hyperventilation

2. Decreased pulmonary blood flow.

10.

Management of spell:
Initial
1. Knee to chest or squatting position.
2. High flow oxygen
3. Morphine 0.1 mg/kg i.m./Sc to suppress respiratory
centre and to break hyperpnoea
4. Propranolol .1/kg slow IV to reduce heart rate.
5. Correction of acidosis
If prolonged
1. IV phenyephrine bolus 10mcg/kg IV to increase

systemic venous pressure

8.
9.

ECG:
CXR:

2. Ketamine 1-3 mg/Kg IV over 60 secs to increase

systemic vascular resistance and to provide sedation
3. Intravenous fluids 10 ml/kg bolus followed by
maintenance fluids.
4. Sodium bicarbonate 23 mmol/kg i.v. (ensure
adequate ventilation).
1. Rt axis deviation 2. RVH
3. BVH in acyanotic form
1. Heart smaller than normal ( small tumultuous heart)
2. Pulmonary oligemia; black lung in PS
3. Concave PA segment; upturned apex due to RVH leading to boot
shape (Coeur en Sabot)
4. RA enlargement in 25%
5. Rt. aortic arch in 25%

9. ECHO:

25
1. Prenatal diagnosis of tetralogy of Fallot may be made readily by fetal
echocardiography
2. ECHO findings postnally include the malaligned conoventricular septal
defect, the anterior deviation of the conal septum, the level or levels of
right ventricular outflow tract obstruction, and the dextroposed,
overriding aorta.
10. Cath:
1. Not needed as ECHO is more informative
2. Balloon dilation of a stenotic pulmonary valve may be useful as a
palliative step
11. Natural History and complications:
1. Infundibular stenosis is progressive and clinical worsening occurs with age
2. Polycythemia is progressive and so cyanosis deepens with age
3. Anemia supervenes
4. Recurrent cyanotic spells; spell can be fatal in infants
5. Growth retardation and cognitive impairment due to chronic tissue
hypoxia
6. Cerebral abscess; bacteria entering thrugh skin breaks or pustules reach
systemic circulation, RA, RV and bypass lungs through overriding aorta
(no pulmonary filtering ) and can rech crebram to develop into an abcess.
7. Cerebral vascular thrombosis and pigment gallstones due to polycythemia
8. Infective endocarditis is a life long risk.
9. Coagulopathy due to polycythemia
10.Progressive aortic root dilation and resulting aortic valve insufficiency
develop in some
12. Management of TOF:
1. If newborns with critical right ventricular outflow obstruction is PDA
prostaglandin E1 (alprostadil) infusion to maintain ductal patency
2. Iron therapy: iron deficiency state should be detected and treated. Irondeficient children are more susceptible to cerebrovascular complications.
1. Surgical:
i. Emergency peocedures for spell: Balloon dilatation of the right
ventricular outflow tract and pulmonary valve
ii. Palliative:
1. Indication:
a. TOF and pulmonary atresia; Unfavorable coronary
artery anatomy; Infants younger than 3 to 4 months;
< 2.5 kg wt
2. Blalock-Tausig procedure: anastomosis between subclavian
and pulmonary artery of opposite side of aortic arch
3. Modified Blalock-Tausig: interposition of (Gore-Tex) shunt
between subclavian and PA in the same side of aortic arch
4. The Waterston shunt, anastomosed between the ascending
aorta and the right PA, is no longer performed
5. The Potts operation, anastomosed between the descending
aorta and the left PA, is no longer performed either.
iii. Complete repair:
1. Indication cyanotic spell ;
2. Preferable age: 1-2 yrs
3. Early repair helps decrease RVH and normal growth of PA
4. Procedure:
a. Patch closure of VSD

26
b. Resection of Infundibular tissue
c. Pulmonary valvotomy
5. PO complications:
a. Pulmonary valve regurgitation
b. CHF
c. Right bundle branch block (RBBB)
d. Complete heart block-rare
6. Mortality: 2-3 %
TOTAL ANOMALOUS PULMONARY VENOUS RETURN:
1. 1% of CHDs; male: female: 4:1
2. Pathology:
a. Pulmonary veins drain into RA or other systemic veins:
i. Rt. SVC in 50%
ii. Coronary sinus 20%
iii. IVC or its branches in 20%
iv. Mixed 10%
b. ASD or PFO
c. PH
d. LV hypoplasia
3. Clinical:
e. Mild cyanosis
f. Recurrent LRI
g. CHF
h. CVS:
i. Quadruple rhythm
ii. S2 widely split
iii. P2 Fixed and loud
4. CXR: snowman or figure of 8 appearance
5. Treatment: surgical redirection
TRICUSPID ATRESIA:
1. Pathology:
a. Absence of tricuspid valve
b. RV hypoplasia
c. ASD, VSD or PDA
d. TGA in 30%
e. PS with PA hypoplasia in 50%
2. Clinical:
a. Cyanosis from birth
b. Hypoxic spells
c. S2 single
d. Systolic murmur of VSD
3. CXR:
a. RA hypertrophy
b. LV hypertrophy
c. Pulmonary oligemia
d. Concave PA segment- boot shape
4. Echo: Absence of tricuspid orifice
Epsteins Anomaly:
1. 1% incidence
2. Downward displacement of septal and posterior leaflets of tricuspid
valve into RV-atrialization

27
3.
4.
5.
6.
7.
8.

Tricuspid regurgitation
RA hypertrophy
ASD or PFO with Rt. to Lt. shunt
Fibrosis of Rt. ventricular valves
WPW syndrome
Clinical:
i. Cyanosis with CCF
ii. Episodes of supra ventricular tachycardia (SVT)
iii. Triple or quadruple rhythm- S1, Split S2, S3, S4 could be heard
iv. Systolic murmur of TR
v. Hepatomegaly
9. CXR: balloon shaped heart with pulmonary oligemia
10.ECG: RBBB; RAH; WPW in 15%
11.Echo: tricuspid valve is displaced toward the apex
12.
Surgery: BT shunt; biventricular repair
Truncus Arteriosus:
1.
2.
3.
4.
5.
6.
7.
8.

Single arterial trunk leads to pulmonary, systemic and coronary circulation.

Truncal valve is incompetent
VSD
Rt. aortic arch in 30%
Digeorge syndrome with hypocalcemia in 33%
Features: cyanosis; CCF
CXR: Cardiomegaly with PH
Echo: single great artery
Persistent Pulmonary Hypertension of NB (PPHN)
1. 1 in 1500 NB
2. Rt to LT. shunt
3. Cyanosis due to PFO or PDA
4. No CHD
5. Causes:
a. Meconium aspiration
b. Hyaline membrane disease
c. birth asphyxia
d. Polycythemia
e. Sepsis
6. Clinical:
a. Grunting and cyanosis in < 12 hrs of life
b. S2 single and loud
c. Gallop due to myocardial dysfunction
7. Management:
a. Mechanical ventilation with 02
b. Induction of alkalosis with O2 and bicarbonate infusion
c. Dopamine infusion
d. Nitric oxide- selective pulmonary vasodilator
RHEUMATIC FEVER

Definition:
It is considered to be a delayed autoimmune reaction in genetically predisposed
individuals to group A, -hemolytic, streptococcal pharyngitis.
Epidemiology:

28
1. Age incidence: 5-15 years; Peak incidence around 8 years; School children
are susceptible population; Prevalence = 5.3/ 1000 school children;
rheumatic heart disease remains the most common form of acquired
heart disease in all age groups, accounting for as much as 50% of all
cardiovascular disease
2. Sex Incidence: Male: female equally affected
3. Predisposing factors:
a. Genetic predisposition-HLA DR-3
b. Immune response gene (B-cell alloantigen (D8/17)
c. Poor socioeconomic status
d. Overcrowding and poor housing
e. Poor sanitation and hygiene
f.

Malnutrition

g. Lack of health facility

4. Etiology
1. Group A b hemolytic streptococcus infection
2. Only pharyngeal infection predispose to Rheumatic fever; streptococcal
carrier state & skin infection does not produce Rh fever;
3. Common strains are M 1, 3, 5,6,18 and 24
5. Etiopathogenesis
1. Sensitization of B lymphocytes by streptococcal antigens
2. Formation of anti-streptococcal antibodies in the host
3. Formation of immune complexes that cross react with myocardial
sarcolemma proteins
4. Myocardial and valvular inflammation
5. Two theories:
a. The cytotoxicity theory: streptolysin O, has a direct cytotoxic effect
on cardiac cells; inability to explain the latent period between GAS
pharyngitis and the onset of acute rheumatic fever

29
b. Molecular mimicry: M proteins (super antigens) share epitopes with
human tropomyosin and myosin.
6.

Clinical features

1. MIGRATORY POLYARTHRITIS:
1. Arthritis occurs in about 75% of patients and involves larger joints, the
knees, ankles, wrists, and elbows. Involvement of the spine, small joints of
the hands and feet, or hips is uncommon.
2. The joint involvement is characteristically migratory in nature;
3. A dramatic response to even small doses of salicylates is another
characteristic feature of the arthritis, and the absence of such a response
should suggest an alternative diagnosis.
4. The arthritis heals completely without sequelae. (RF licks the joint but
bites the heart)
5. There is often an inverse relationship between the severity of arthritis and
the severity of cardiac involvement.
6. DD of polyarthritis:
N
o.
1

Condition

Differentiating signs/symptoms

Septic arthritis

Usually only one joint involved; not migratory;

patient looks toxic.

Juvenile Rheumatoid
arthritis

Lasts longer than 6 weeks; may not have joint

pain; eye inflammation may be present. Light pink
rash in systemic form.

Poststreptococcal
reactive arthritis

nonmigratory ; Small peripheral joints Few may

develop RF

Lyme disease

erythema migrans; influenza-like symptoms;

Bell's palsy;

Sickle cell anaemia

Family history; signs and symptoms of anaemia;

not usually febrile unless infection has precipitated
crisis.

30
6
Janeway lesions, Osler nodes, and splinter
haemorrhages.

Infective
endocarditis
7
Leukemia

bone pain;

8
First metacarpophalangeal joint; flaky red skin
over affected joint.

Gout and
pseudogout
9
SLE

Malar 'butterfly' rash; joint pain typically affects

hand and wrist;

2. Carditis
1. Carditis occurs in about 50-60% of all cases
2. It is pancarditis-pericardium, myocardium, endocrdium or valves; Cardiac
involvement during acute rheumatic fever varies in severity from
fulminant, potentially fatal exudative pancarditis to mild, transient cardiac
involvement. Endocarditis (valvulitis) is a universal finding in rheumatic
carditis, whereas the presence of pericarditis or myocarditis is variable.
3. Valvular insufficiency is characteristic of both acute and convalescent
stages of acute rheumatic fever, whereas valvular stenosis usually
appears several years or even decades after the acute illness
4. In developing countries, mitral stenosis and aortic stenosis may develop
sooner after acute rheumatic fever and can occur in young children.
5. Mitral valve most commonly affected and more in female
6. Aortic valve (incompetence) more in male
7. Pulmonary and tricuspid valves less common
Clinical features of carditis:
1. Acute rheumatic carditis usually presents as tachycardia and changing
cardiac murmurs
2. Severe rheumatic carditis can result in cardiomegaly and congestive heart
failure with hepatomegaly and peripheral and pulmonary edema

31
3. Mitral regurgitation is characterized by a high-pitched apical holosystolic
murmur radiating to the axilla. In patients with significant mitral
regurgitation, this may be associated with an apical mid-diastolic murmur
of relative mitral stenosis due to thickened mitral valve (Carey Cooms
murmur). Aortic insufficiency is characterized by a high-pitched
decrescendo diastolic murmur at the upper left sternal border.
4. Although controversial, subclinical valvular regurgitation is not currently
accepted as either a major or minor Jones criterion
5. Recurrent attacks of acute rheumatic fever are associated with high rates
of carditis.
6. The major consequence of acute rheumatic carditis is chronic, progressive
valvular disease, particularly valvular stenosis.
7. Echocardiographic findings include pericardial effusion, decreased
ventricular contractility, and aortic and/or mitral regurgitation.
8. Pericarditis: pericardial rub with effusion
8. Sydenham Chorea
1. Epidemiology:
1. It occurs in about 10-15% of patients with acute rheumatic fever
and usually presents as an isolated, frequently subtle,
neurologic behavior disorder.
2. The latent period from acute GAS infection to chorea is usually
longer than for arthritis or carditis and can be months.
3. Onset can be insidious, with symptoms being present for
several months before recognition.
4. Rare after the age of 20 years.
5. Occurs more in females,
6. May last up to 3 months
7. Recurrences common
8. Does not lead to permanent neurologic sequelae .
9. Carditis can develop later
2.

Clinical features:

32
1. Emotional lability, incoordination, poor school performance,
uncontrollable movements, and facial grimacing, exacerbated
by stress and disappearing with sleep, are characteristic.
2. Inattentive to schoolwork, fidgety; clumsy; drops objects
3. illegible hand writing
4. Ataxia;
5. Slurred speech
6. Muscular weakness
7. Chorea occasionally is unilateral.
3. Clinical signs:
1. Milkmaids grip (irregular contractions of the muscles of the
hands while squeezing the examiners fingers),
2. Pronator sign: pronation of the hands when the patients arms
are extended,
3. Adder sign: wormian darting movements of the tongue upon
protrusion,
4. Handwriting: micrographia
5. Pendular knee jerk
6. Dorsum of hands on extension assumes a spoon or dish
configuration
4. DD of Chorea:
1. Atypical seizure- EEG
2. SLE - positive ANA titres
3. Drug intoxication- Drug screen
4. Familiar chorea long history
5. Hormonally induced chorea : chorea gravidorum and Oral pill
6. Wilsons disease - Decreased serum ceruloplasmin level. KF ring
5. Treatment of Sydenham Chorea.
1.Anti-inflammatory agents are usually not indicated.

33
2.Phenobarbital (1632 mg every 68 hr po) is the drug of choice.
3.If phenobarbital is ineffective, then haloperidol (0.010.03
mg/kg/24 hr divided bid po) or chlorpromazine (0.5 mg/kg every
46 hr po) should be initiated
10.

Erythema marginatum
1. In 7 %
2. Macular
3. Serpigenous
4. Erythematous
5. Central clearance
6. Trunk and limbs involved
7. Face spared
8. Not well seen in our population
9. Carditis often associated

11.

Subcutaneous nodules
1. In 5 %
2. Occur in severe cases. There is a correlation between the presence
of these nodules and significant rheumatic heart disease
3. Seen over elbows, joints, scalps, spinal column
4. Size: few mm to 1 cm
5. Non tender
6. Movable under the skin
7. DD: SLE and rheumatoid arthritis.

12. Abdominal Pain in Rh Fever:

1. At intervals in the past, children were hospitalized for abdominal pain with
fever and ended up with abdominal exploration for appendicitis, after
which it was discovered that the child had acute rheumatic fever.
2. Whether this abdominal pain is a consequence of pericardial effusion or
inflammation of the abdominal serous surfaces is not clear. However, it
has occurred often enough to be reasonably well documented.

34
13.

Minor Manifestations;
1. The 2 clinical minor manifestations are arthralgia (in the absence of
polyarthritis as a major criterion) and fever (typically temperature 102F
and occurring early in the course of illness).
2. The 2 laboratory minor manifestations are elevated acute-phase reactants
(e.g., C-reactive protein, erythrocyte sedimentation rate) and prolonged
PR interval on electrocardiogram (1st degree heart block).

14.

Diagnosis of Rheumatic Fever:

Modified Jones Criteria: 1992
1. Because no clinical or laboratory finding is pathognomonic for acute
rheumatic fever, T. Duckett Jones in 1944 proposed guidelines to aid in
diagnosis and to limit overdiagnosis.
2. The Jones criteria was revised in 1992 by the American Heart
Association (AHA) for the diagnosis of the initial attack of acute rheumatic
fever and not for recurrences.
3. There are 5 major and 4 minor criteria and an absolute requirement for
evidence (microbiologic or serologic) of recent GAS infection.
4. The diagnosis of acute rheumatic fever can be established by the Jones
criteria when a patient fulfils 2 major criteria or 1 major and 2 minor
criteria and meets the absolute requirement (essential criteria).
5. There are 3 circumstances in which the diagnosis of acute rheumatic fever
can be made without strict adherence to the Jones criteria.
a. Chorea may occur as the only manifestation of acute rheumatic
fever.
b. Indolent carditis may be the only manifestation in patients who 1st
come to medical attention months after the onset of acute
rheumatic fever.
c. Most patients with recurrences of acute rheumatic fever may not
fulfil the Jones criteria.
GUIDELINES FOR THE DIAGNOSIS OF INITIAL ATTACK OF RHEUMATIC FEVER (JONES
CRITERIA, UPDATED 1992)

Major

Minor

Supporting evidence of

Manifestations

Manifestations

Antecedent group a

1. Carditis
2. Polyarthritis
3. Erythema
marginatum
4. Subcutaneous
nodules
5. Chorea

Clinical criteria :
1. Arthralgia
2. Fever
Laboratory features:
3. Elevated acute phase
4. reactants:
5. Erythrocyte
6. sedimentation rate
7. C-reactive protein

Streptococcal infection
within the last 45 days
1. Positive throat
culture or rapid
streptococcal antigen
test
2. Elevated or
increasing
3. streptococcal
antibody titer

35

Prolonged PR interval

6. Original Jones Criteria - 1944

i. Major:
1. Carditis
2. Arthralgia
3. Chorea
4. Subcute. Nodules
5. Hx of prev ARF
6. Rheumatic H.D.
ii. Minor :
1. Fever
2. Abdominal Pain
3. Precordial Pain
4. E. Marginatum
5. Epistaxis
6. Pul. Findings
7. Lab findings - ESR, WBCs
8. Anemia,
9. ECG changes.
15.
Recent diagnostic criteria for Acute Rheumatic fever in children.
(WHO): 20022003
Diagnostic categories
1. Primary episode of RF

2. Recurrent attack of RF in a
patient without
established rheumatic
heart disease.
3. Recurrent attack of RF in a
patient with established
rheumatic heart disease
4. Rheumatic chorea.
5. Insidious onset rheumatic
carditis
6. Chronic valve lesions of
RHD (patients presenting
for the first time with
established valve disease)

14.

Criteria
Two major or one major and two minor
manifestations plus evidence of a preceding
group A streptococcal infection
Two major or one major and two minor b
manifestations plus evidence of a preceding
group A streptococcal infection
Two minor manifestations plus evidence of
a preceding group A streptococcal infection.
Other major manifestations or evidence of
group A streptococcal infection not required.
Do not require any other criteria to be diagnosed
as having rheumatic heart disease

Evaluation of Rheumatic Fever:

1. Echocardiography: ECHO based diagnostyic criteria is becoming more
popular than Jones.
1.
Identifies the size of atria and ventricles, valvular thickening,
leaflet prolapse, coaptation failure, restriction of leaflet motility, and
ventricular dysfunction

36
2.
Serves to establish the diagnosis of mitral and/or aortic
insufficiency
2. Lab tests:
1.
Throat swap
2.
ASO titers of at least 333 Todd units in children and 250 Todd
units in adults are considered elevated. A single low ASO titer does not
exclude acute rheumatic fever.
3.
The antideoxyribonclease B test: titers of 240 Todd units or
greater in children are positive.
4.
Rapid antigen detection tests
15.
Treatment of Rhematic Fever
1. General measures:
i. Bed rest and monitored for evidence of carditis.
2. Eradication of streptococci:
i. The drug of choice is penicillin;
ii. Duration of treatment is 10 days
iii. Orally administered penicillin -penicillin V or penicillin G
Erythromycin, or a Single IM benzathine penicillin
iv. First-generation cephalosporins have also been used successfully
3. Anti-Inflammatory Therapy.
i. For undiagnosed RF use paracetamol for joint pain
ii. Polyarthritis with carditis and without cardiomegaly or congestive
heart failure:
1. aspirin is 100 mg/kg/day in 4 divided doses PO for 35 days,
for 2 weeks followed by
2. 75 mg/kg/day in 4 divided doses PO for 4 wk.
iii. For carditis with cardiomegaly or congestive heart failure:
1. Prednisone 2 mg/kg/day in 4 divided doses for 23 wk
followed by
2. Tapering of the steroid by 5 mg/24 hr every 23 days until
adequate response.
3. With tapering , aspirin should be started at 75 mg/kg/day in
4 divided doses for 6 wk
4. Supportive therapies:
i. For patients with moderate to severe carditis include digoxin, fluid
and salt restriction, diuretics, and oxygen.
ii. The cardiac toxicity of digoxin is enhanced with myocarditis.
16. Prophylaxis for Rheumatic fever.
I.
Primary prophylaxis:
a. Adequate antibiotic therapy of group A streptococcal upper
respiratory tract infections to prevent an initial attack of acute RF.
a. Recommended treatment for strep.pharyngitis:
Antibiotic
Benzathine
benzylpenicilli
n

Dose
Single intramuscular injection
1200000 units intramuscularly; 600000 units for children
weighing <27kg

Phenoxymeth
yl penicillin
(Penicillin V)

Orally 24 times/day for 10 full days

Children: 250 mg bid or tid.
Adolescents or adults: 250mg tid
or qid, or 500mg bid.
Orally 23 times/day for 10 full days
2550mg/kg/day in three doses. Total adult dose is.

Amoxicillin

37

Firstgeneration
cephalosporin
sc
Erythromycin

II.

7501500mg/day.
Orally 23 times/day for 10 full days; Dose varies with
agent.

mg/kg/day in divided dose orally 4 times/day for 10 full

days

Secondary prevention of rheumatic fever: Antibiotics used in

secondary prophylaxis of RF
Antibiotic
Benzathine
Benzylpenicillin.
Penicillin V.
Sulfonamide
(e.g. sulfadiazine

Dose
Single intramuscular injection every 34 weeks
For adults and children 30kg in weight: 1200000
units. For children < 30kg in weight: 600000 units.
Oral. 250mg twice daily.
Oral. For adults and children 30kg in, weight:
gram daily. For children <30kg in weight: 500mg

daily
sulfadoxine, sulfisoxazole).
Erythromycin.

.
Oral. 250mg twice daily.

Duration of secondary prophylaxis:

Category of patient
Patient without proven
carditis.

Duration of prophylaxis
For 5 years after the last attack, or until 18 years
of age (whichever is longer).

Patient with carditis

For 10 years after the last attack, or at least until

25 years of age (whichever is longer).
Lifelong.
Lifelong.

More severe valvular disease

After valve surgery.

17.

Rheumatic fever: Indian context

1. Two studies published in the 1960s showed that the presentation of
rheumatic heart disease is different in developing countries. These studies
reported:
1. Symptomatic heart disease at younger age of onset
2. An increased rate of rheumatic valvular heart disease among
individuals younger than 20 years
3. Less interval between the onset of symptoms and symptomatic
rheumatic fever
4. Severe mitral stenosis and its complications within a short duration of
onset of disease.
5. In addition, mitral value calcification, atrial fibrillation, and
thromboembolic complications are less common in developing
countries than in developed countries.
6. The reasons for different presentation of this disease in developing
countries are not clear, but may be due to:
1. Younger age of onset,
2. Heavy infetion with streptococcus and increased antigenic load
3. Malnutrition
4. Recurrent attacks of rheumatic fever,

38
5. Low socioeconomic status,
6. Inadequate penicillin prophylaxis,
7. Increased genetic susceptibility linking to HLA.
7. The clinical profile of acute rheumatic fever in India also differs as

follows:
1. High prevalence of carditis, congestive heart failure, and
mortality. This high incidence arises because in developing
countries with limited health-care facilities patients continue to
be physically active during the long pre-admission period.
2. Erythema marginatum is probably never observed, while
subcutaneous nodules and chorea are uncommon.
3. Polyarthritis more often occurs as migratory arthralgia than as
swelling of the joints
18.
NON- SUPPURATIVE COMPLICATIONS OF GR A BETA HEMOLYTIC
STREPTOCOCCI.
a. Rheumatic fever
a. Carditis
b. Poly Arthritis
c.

Subcutaneous nodules

d. Erthyme multiforme
e. Chorea
b. Poststreptococcal glomerulonephritis
a. nephritogenic strains of GAS (types 12 and 49)
c. Central Nervous System Diseases:
i.
Isolated Chorea: is the neurologic manifestation of rheumatic
fever.
ii.

Reports of obsessive-compulsive disorder (OCD), tic disorders, may

occur in association with group A beta-hemolytic streptococcal
infections

iii.

Pediatric Autoimmune Neuropsychiatric Disorders

Associated With Streptococcus Pyogenes (PANDAS).
1. A group of neuropsychiatric disorders (particularly obsessivecompulsive disorders, tic disorders, and Tourette syndrome) for
which a possible relationship with GAS infections has been
suggested.
2. Because of the proposed autoimmune mechanism, it has also
been suggested that these patients may benefit from
immunoregulatory therapy such as plasma exchange or
intravenous immunoglobulin therapy.

39
d. Poststreptococcal reactive arthritis:
a. Onset of acute arthritis following an episode of GAS pharyngitis
without any other Jones criteria is considered as reactive arthritis
b. There is still considerable debate about whether this entity
represents a distinct syndrome or is a manifestation of acute
rheumatic fever.
c. it may involve small peripheral joints as well as the axial skeleton
and is typically nonmigratory.
d. The incubation is < 10 days in contrast to ARF
e. It does not respond dramatically to therapy with aspirin or other
nonsteroidal anti-inflammatory agents.
f. A small proportion of patients with poststreptococcal reactive
arthritis may go on to develop valvular heart disease.
RHEUMATIC HEART DISEASES:
Mitral Stenosis
1. Rare in young children as it takes 5-10 yrs after Rheumatic fever
2. Congenital MS: very rare
3. Rheumatic MS pathology:
1. Thickening of leaflets
2. Fusion of commissures
4. Hemodynamics:
1. LA enlargement
2. Pulmonary venous hypertension & Pulmonary arterial hypertension
3. RVH
5. Symptoms:
1. Dyspnoea;
2. orthopnoea;
3. nocturnal dyspnoea;
4. palpitation
6. Clinical features:
1. Malor flush,
2. parasternal heave;
3. raised JVP;
4. Loud S1
5. S2 split with P2 louder
6. Opening snap
7. Diastolic rumble at apex with presystolic crescendo murmur
8. Diastolic rumble Rt. Sternal border ( Graham Steel) due to functional
PR
7. ECG: RT. Axis deviation; LAH; RVH; Atrial fibrillation (rare)
8. CXR:
i. LAH, RVH, prominent PA; Lung venous congestion;
ii. Kerley B lines: horizontal lines in costophrenic angles due to
interstitial oedema
iii. Trivial enlargement of the transverse diameter of the heart.
iv. Left atrium causes double outline- shadow in shadow Left atrial
appendage is dilated, causing a prominence of the left borderstraightening of left border
v.
Upper lobe vessels larger than lower lobe vessels, that is, upper lobe
blood diversion.
9. ECHO: valve lesions; dilated LA; slow movement of MV
10.Signs of Severity:
1. Duration of murmurs (longer is severe)

40
2. S1 -S interval (shorter is severe)
DD:
1. Cor triatriatum: double chamber in LA with obstruction to
pulmonary venous return and PH
2. Stenosis of pulmonary veins
3. Hypoplastic Lt. heart syndrome
4. Lt. atrial myxoma
12.Natural History:
1. Stenosis is progressive with age
2. Symptoms aggravate
3. Atrial fibrillation
4. Thrombo embolism
5. SBE
6. Haemoptysis due to pulmonary congestion
13.Management:
1. Digoxin; diuretics
2. Balloon dilatation of MV
3. IV procainamide for AF
4. Anticoagulation for chronic AF-embolism
5. Dental hygiene; SBE prophylaxis
6. Exercise restriction
7. Penicillin prophylaxis
14.Surgical:
Indications: symptoms of angina, syncope, effort intolerance, PA
pressure>50 mmHg; failed balloon dilatation; Recurrent AF
1. Resection of supravalvular mitral ring
2. Splitting of fused chordate
3. Commissurotomy
4. Valve replacement
5. Fenestration for parachute MV
15.Post op. Complications: CCF; Embolization; bleeding
11.

1.
2.
3.
4.

5.

6.
7.
8.
9.

Mitral Regurgitation:
More common than MS
Mostly congenital in association with AV canal defect
Rarely Rheumatic in origin due to fibrosis of mitral leaflets
Clinical:
1. Mild MR: asymptomatic
2. Fatigue due to poor cardiac output
3. Palpitation due to atrial fibrillation
Signs:
1. LAH; LVH; and PH
2. Heaving apical impulse
3. S1 normal or soft
4. S2 widely split due to shortening of LV ejection and early closure of
Atrial valve
5. Pan systolic murmur at apex conducted to Lt.axilla
6. Short diastolic rumble at apex (relative MS)
ECG: normal in mild; LVH, LAH, AF may be present
CXR: LAH, LVH, pulmonary venous congestion
Echo: Dilated LA, LV and regurgitant get
Management:
1. SBE prophylaxis

41
2. Exercise restriction
3. Diuretics and digoxin
4. Surgical:
i. Indications: symptomatic pt; LV ejection fraction <.6;
intractable CCF; PH
ii. Mitral valve replacement
iii. Repair of cleft leaflet
iv. Annuloplasty
v. Commissuroplasty
Aortic regurgitation:
1. Causes:
1. Mostly congenital: bicuspid valve; a
2. After balloon dilatation
3. Sub aortic dilatation in Marfan and Ehlers Danlos
2. Clinical:
1. Mild asymptomatic
2. Effort intolerance
3. Physical Findings:
i. Hyper dynamic precardium
ii. Apical impulse down and out & heaving
iii. Diastolic thrill in 3rd Lt. Intercostal space
iv. Wide pulse pressure; water hammer pulse
v. S1 increased; S2 normal or single
vi. High pitched diastolic decrescendo murmur at 3 rd Lt. Intercostal
space; better heard on sitting leaning forward
vii. Systolic murmur 2nd Rt. intercostal space due to relative AS
viii. Mid diastolic mitral rumble at apex (Austin Flint)
4. Signs of Severity:
a. Duration of murmur longer is severe
b. Systolic ejection flow murmur
c. Austin flint
d. Reduced diastolic BP
5. ECG: LVH, LAH.
6. CXR: LV, dilated ascending aorta, prominent aortic knob, LVF with
pulmonary congestion.
7. Echo: LV dilatation; aortic regurgitant stream
8. Natural History:
i. Mild: asymptomatic
ii. Angina, CCF, Premature ventricular contractions
iii. SBE
9. Management:
i. Medical:
1. Oral hygiene
2. SBE prophylaxis
3. Exercise restriction
4. ACE inhibitor to reduce LVH
5. Digoxin; diuretics
ii. Surgical:
1. Valvuloplasty
2. Valve replacement
3. Ross procedure: Intact pulmonary valve grafted to aorta
with coronary implantation
iii. Post-operative Complications: CCF; embolism; endocarditis

42

Mitral Valve Prolapse:

1. Mitral valve prolapse results from an abnormal mitral valve mechanism that
causes billowing of one or both mitral leaflets, especially the posterior cusp,
into the left atrium toward the end of systole.
2. The abnormality is predominantly congenital but may not be recognized until
adolescence or adulthood. Mitral valve prolapse is usually sporadic, is more
common in girls, and may be inherited as an autosomal dominant trait with
variable expression. Male: female 1:2
3. 2-5 %; increasing incidence in adults;
4. It is common in patients with Marfan syndrome, straight back syndrome,
pectus excavatum, scoliosis, Ehlers-Danlos syndrome, osteogenesis
imperfecta, and pseudoxanthoma elasticum. Associated with ASD, VSD, and
Epstein in some.
5. Pathology:
a. Thick and redundant leaflet mostly posterior one; bulge into mitral
annulus due to myxomatous degeneration
b. Displacement of an abnormally thickened mitral valve leaflet into the
left atrium during systole.
c. There are various types of MVP, broadly classified as classic and
nonclassic. In its nonclassic form, MVP carries a low risk of
complications. In severe cases of classic MVP, complications include
mitral regurgitation, infective endocarditis, congestive heart failure,
andin rare circumstancescardiac arrest, usually resulting in sudden
death
d. Thoracic gage may have abnormalities like pectus excavatum, straight
back syndrome ( reduced anteroposterior diameter of chest) &
scoliosis
6. Clinical:
a. Asymptomatic in most people.
b. Some may have non exertional chest pain, palpitation, syncope due to
tension in papillary muscle
c. Ausculation: There may be an apical murmur in late systolic and may
be preceded by a click, but these signs may vary in the same patient
and, at times, only a mid diastolic click is audible. In the standing or
sitting position, the click may occur earlier in systole, and the murmur
may be more prominent in late systole.
d. Arrhythmias may occur and are primarily unifocal or multifocal
premature ventricular contractions.
7. ECG: may be normal; may show biphasic T waves; WPW incidence slightly
more; RBBB occasional; LVH or LAH rare.
8. CXR: normal; LAH if there is MI
9. Echo: may be normal in children; progress of lesion seen in adults; prolapse
visualized; The echocardiogram shows a characteristic posterior movement
of the posterior mitral leaflet during mid- or late systole or demonstrates
pansystolic prolapse of both the anterior and posterior mitral leaflets
10.Natural History: no symptoms in childhood; rarely death due to arrhythmia;
progressive to MI;
11.Complications:
a. Adults (men more often than women) with mitral valve prolapse are at
increased risk for cardiovascular complications (sudden death,

43
arrhythmia, cerebrovascular accidents, progressive valve dilatation,
heart failure, and endocarditis) in the presence of thickened (>5 mm)
and redundant mitral valve leaflets.
b. Risk factors for morbidity also include poor left ventricular function,
moderate to severe mitral regurgitation, and left atrial enlargement.
12.Management:
a. This lesion is not progressive in childhood, and specific therapy is not
indicated
b. No treatment for asymptomatic patient
c. B-blockers for palpitation, arrhythmias, chest pain.
d. Surgery for MR
Cardiomyopathy:
1. Disease of heart muscle not associated with congenital, valvular, coronary
defects or systemic disorders.
2. Three types:
No
1

Type
Hypertroph
ic

Etiology
Autosomal
dominant

Dilated or
congestive

1. Toxic
2. metabolic
3. Infective

Restrictive

1. amyloid
infiltration
2.
heamochromotosis

Features
1. Massive Lt. ventricular
hypertrophy With outflow
tract obstruction
2. small ventricular cavity
Lt atrial enlargement
3. normal contractility
4. Impaired relaxation and
ventricular filling
5. pulmonary congestion
1. Decreased contractility
2. dilated ventricles

Syndromes
1. Leopard
syndrome
2. NB of diabetic
mother

1. dilatation of atria
2. restricted diastolic filling
of ventricles

1. Hypertrophic:
a. Features:
1. Family history in 30-60%
2. Massive Lt. Ventricular hypertrophy with outflow tract obstruction;
Small ventricular cavity Lt atrial enlargement
3. Normal contractility
4. Impaired relaxation and ventricular filling
5. Pulmonary congestion
6. LVOT varies from time to time and hence changing murmurs
7. More obstruction during late systole and hence late systolic
murmur with sharp upstroke in pulse wave
b. Symptoms:
1. Anginal pain
2. Near syncope or syncope
3.Arrhythmias due to narrowing of anterior descending
coronary artery
4. Sudden death due to ventricular fibrillation
5. Easy fatigability , dyspnoea, palpitation
c. Physical findings:

44
1.
2.
3.
4.

Sharp upstroke in arterial pulse

LV lift at apex
Systolic thrill at apes and Lt. Sternal border
Holosystolic murmur of MR (varies each time)

d. ECG:
1. LVH
2. ST-T changes
3. Deep Q wave due to septal hypertrophy
4. Absent R wave in Lt. Precardial leeds
5. Occasional giant T waves
6. Arrhythmias
7. A-V block
e. CXR: LVH; globular heart; pulmonary vascularity normal
f. ECHO:
1. Concentric hypertrophy
2. Localised segmental hypertrophy
3. Asymmetrical hypertrophy
4. Increased LV wall thickness >15 mm
5. Diastolic dysfunction
6. LVOT obstruction
g. Natural history:
1. Obstruction may be stable or progressive
2. Sudden death in 10-35 yrs in 4-6 %
3. AF leads to CVA & CCF
4. SBE
5. Pregnancy is usually tolerated
h. Management:
1. Moderate restriction of physical activity
2. Digoxin contra indicated as increases obstruction
3. SBE prophylaxis
4. Screen 1st degree relatives
5. Annual evaluation
6. B-blocker and calcium channel blockers (verapamil) reduce the
obstruction, angina and arrhythmia
7. Morrows myotomy/ myectomy: excision of a rectangular
ventricular septal muscle
8. Percutaneous alcohol septal ablation: reaching through septal
perforator branch of ant. descending artery
9. Implantable cardioverter defibrillator to prevent sudden death
due to arrhythmias.
10.Arrhythmias:
a. Propranolol for ventricular arrhythmias
b. Electrical cardioversion or amiodarone for AF
11.Mitral replacement
Infants of Diabetic mother:
6-9 % has con. defects: anencephaly, myelo meningocele, sacral agenesis,
Cardiomyopathy and PPHN
1. CHD includes VSD, TGA, TA, Tricuspid atresia, COA
2. Hypertrophic Cardiomyopathy may resolve over 6-12 mounts
Neonates: Hypertrophic Cardiomyopathy may follow foetal distress or birth
asphyxia; resolve in 1-5 months
Dilated or congestive Cardiomyopathy:
Aetiology:

45
1. Idiopathic
2. Familial
3. Infectious(viral)
4. Metabolic: hypothyroidism; diabetes, muco polysachharidosis etc
Pathology:
1. 4 chamber dilatation
2. Pulmonary and systemic embolism common
3. Early CCF
4. S3 prominent
Natural history: death in 4 years
Restrictive Cardiomyopathy:
1. Rare; 5 % of Cardiomyopathy
2. Idiopathic or associated with scleroderma, amyloidosis, sarcoidosis,
mucopoly, malignancy and radiotherapy etc.
3. Dilated atria, normal ventricles but impaired filling due to stiff walls;
resembles constrictive pericarditis.
4. Echo: diastolic dysfunction CXR: Cardiomegaly

1.
2.

3.
4.
5.
6.

7.

Pulmonary Hypertension:
Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial
pressure and secondary right ventricular failure.
PH is defined as a mean pulmonary artery pressure greater than 25 mmHg at
rest at sea level. A mean pulmonary artery pressure of 8 to 20 mmHg at rest
is considered normal
Systolic pressure in PA : <30 mm Hg
Mean PA pressure is 25 mm Hg
Systolic pressure > 40 mm Hg is pulmonary hypertension
Causes ( Classification) of PH:
1. Left to Right shunt: VSD, PDA and ECD
2. Alveolar hypoxia:
i. Pulmonary parenchymal disease:
1. pneumonia;
2. Interstitial lung disease;
ii. airway obstruction
1. Laryngotracheomalasia
2. Bronchial obstruction
3. Cystic fibrosis
3. Pulmonary venous hypertension:
i. Mitral stenosis,
ii. COA
iii. TAPVR
4. Disease of pulmonary vasculature:
i. PPHN
ii. PPH
5. Others:
i. pulmonary embolism
ii. SLE
iii. Rheumatoid arthritis
iv. Sarcoidosis
v. HIV
Pathophysiology:
Factors increasing PVR:
1. Pulmonary artery stenosis
2. Increase in PA pressure transmitted by LA pressure

46
3. Angiotensin II ( produced by ACE from AI) is pulmonary
vasoconstrictor
4. Less alveolar O2 tension
5. Acidosis
Factors decreasing PVR:
1. Nitric oxide
8. Clinical:
1. Exertional dyspnoea; fatigue; headache
2. Syncope on exertion
3. Cough and wheezing
4. Haemoptysis
9. Physical findings:
1. Cyanosis
2. Prominent neck veins; A- wave prominence
3. apical tap: Left parasternal lift (RVH)
4. Hepatomegaly; ankle edema
5. Early systolic click (sudden opening of P.V. into high pressure artery)
6. Prominent P2 (due to increase force of pulmonary valve closure)
7. Mid systolic ejection murmur (turbulent trans valvular pulmonary flow)
8. Signs of Severity:
1. Murmur of PR
2. Murmur and peripheral signs of TR
3. If advanced pulmonary HYPERTENSION look for signs of RVF
10.
ECG:
1. Rt. axis deviation 2. RVH
3. RAH
11.
CXR:
1. Cardiomegaly with CCF
2. Prominent PA with hilar vessels and clear lung fields
3. Pulmonary oedema
12.Echo: RAH, RVH, and small LV
13.
Natural history:
1. PH is reversible by elimination of cause
2. Infection may exacerbate symptoms PH
3. PPH is progressive- fatal in 2-3 yrs
4. RVF
5. Haemoptysis
6. Arrhythmias
14.
Management:
1. Treat underlying cause
2. Avoid strenuous exercise and high altitudes
3. O2
4. Avoid pregnancy
5. Treat CHF; arrhythmias
6. Prevent RS infections
7. Anticoagulants to prevent micro embolism in pulmonary circulation
8. Nifedipine o.2 mg/kg 8 hrly benefits PPH
9. Prostacyclin infusion
10.Endothelin receptor antagonists (Bosentan, sitaxsentan)
11.Sildenafil- pulmonary vasodilator
12.Nitric oxide inhalation in ARDS and PPHN
13.Atrial septostomy
14.Lung transplantation
MANAGEMENT OF CONGESTIVE CARDIAC FAILURE IN CHILDREN:

47
1. Definition:
It is syndrome in which heart is unable to pump enough blood to the body
to meet its needs, to dispose of systemic or pulmonary venous return
adequately or a combination of all.
2. Important Causes:
a. Volume overload: CHDs like VSD, PDA, ECD ,rheumatic valvular HD,
Anemia
b. Pressure overload: aortic stenosis; Coarctation of aorta; Systemic
hypertension
c. Systemic hypertension: Nephritis
d. Pulmonary hypertension: PPH, PPHN, Sec.PH
e. Tachyarrhythmia: Supraventricular tachycardia
f. Heart block
g. Myocarditis: viral, Kawasaki; Rheumatic
h. Cardiomyopathy: primary , secondary
3. Where CCF is uncommon:
a. Children with tetralogy of Fallot (TOF)
b. Atrial septal defect (ASD)
a. Large left-to-right shunt lesions, such as VSD and PDA, do not cause
CHF before 6 to 8 weeks of age because the pulmonary vascular
resistance does not allow left-to-right shunt until this age.
4. Pathophysiology:
a. Increase in volume overload is compensated by increase in cardiac
output which is no longer possible after a stage
b. Compensatory features:
1. Cardiac hypertrophy
2. The sympathetic nervous system (SNS) provides the rapid response
reflexes, including adrenergic-mediated tachycardia, stimulation of
myocardial contractility, and regional vasoconstriction.
3. Activation of the renin-angiotensin-aldosterone system (RAAS),
which stimulates renal fluid retention to expand vascular volume.
The rise in vascular volume improves cardiac filling and restores
cardiac output
5. Clinical features:
a. Infants:
1. Feeding difficulties
2. Cold sweat on the forehead suggest CHF in infants
3. Heart rates are of more than 160 beats/minute in the neonate
and more than 120 in the older infant.
4. Tachypnea (a resting respiratory rate greater than 60
breaths/minute in the neonate or greater than 40 in the older
infant)
5. wheezing
6. Hepatomegaly
7. Peripheral edema with only very severe heart failure.
8. Cool extremities, weakly palpable pulses, and a low arterial
blood pressure
9. Mottling of the extremities and slow capillary refill
b. In Childhood:
1. Breathlessness
2. Exertional dyspnea
3. Chronic hacking cough,
4. Orthopnea

48
5. Fatigue
6. Malnourished and wasted
c. Signs:
1. Tachycardia and tachypnea.
2. Peripheral vasoconstriction, resulting in coolness, pallor, and
cyanosis of the digits, with poor capillary refill.
3. Venous pulsations visible above the clavicle while the patient is
sitting.
4. Hepatomegaly
5. Peripheral edema with facial puffiness
6. Ascites, pericardial effusion, and, occasionally, hydrothorax.
d. CVS signs:
1. Cardiomegaly. It is usually hyperactive.
2. A third heart sound.
3. Pulsus alternans-alternating strong and weak pulsations,
4. Pulsus paradoxus (a fall in blood pressure on inspiration and a
rise on expiration), secondary to marked swings in
intrapulmonary pressure.
e. Other findings:
1. Proteinuria and high specific gravity of the urine
2. Increase in the blood urea nitrogen and creatinine levels,
secondary to reduced renal blood flow.
3. Hyponatremia, secondary to increased water retention
4. Congestive hepatomegaly and cardiac cirrhosis may lead to
abnormalities in liver enzymes
6. Diagnosis:
a. CXR:
1. X-ray shows cardiac enlargement
2. Interstitial pulmonary edema: kerley's lines, sharp linear
densities in the interlobar septa
b. ECG: points to heart defect
c. Echo:
i. Enlargement of ventricular chambers and impaired LV systolic
function
ii. It helps to determine the cause of CHF.
iii. Echo is also helpfulin serial evaluation of the efficacy of therapy.
a. Biochemical Markers:
a. Severity of heart failure can be assessed by measurement of
natriuretic peptides, serum troponin level, creatinine
phosphokinase
b. Plasma and atrial natriuretic peptides are increased
MANAGEMENT:
i.
Drugs:
DRUG

DOSAGE
DIGOXIN

Digitalization (
Premature: 20 g/kg NOTE: These doses are PO;IV dose is
initially, followed by 75% of po dose
q12h 2)
Full-term neonate (up to 1 mo):2030 g/kg
Infant or child: 2540 g/kg
Maintenance

510 g/kg/day, divided q12h

49
DRUG

DOSAGE

digoxin
DIURETICS
Furosemide (Lasix)

IV:12 mg/dose PO:14 mg/kg/day, divided qdqid

Spironolactone
(Aldactone)

PO:13 mg/kg/day, divided bid or tid

Nesiritide (B-type
natriuretic peptide)

IV:0.0010.03 g/kg/min
ADRENERGIC AGONISTS (ALL IV)

Dobutamine

220 g/kg/min

Dopamine

230 g/kg/min

Isoproterenol

0.010.5 g/kg/min

Epinephrine

0.11.0 g/kg/min

Norepinephrine

0.12.0 g/kg/min
PHOSPHODIESTERASE INHIBITORS (ALL IV)

Amrinone

310 g/kg/min

Milrinone

0.251.0 g/kg/min
AFTERLOAD-REDUCING AGENTS

Captopril (Capoten), Children: 0.12 mg/kg/day q812h

PO
Enalapril (Vasotec),
PO

0.080.5 mg/kg/dose q1224h

Hydralazine
(Apresoline)

IV:0.10.5 mg/kg/dose
PO:0.251.0 mg/kg/dose q68h (maximum, 200 mg/day)

Nitroprusside
(Nipride)

IV:0.58 g/kg/min

Prazosin

PO:0.0050.025 mg/kg/dose q68h (maximum, 0.1

mg/kg/dose)
-ADRENERGIC BLOCKERS

Carvedilol

PO:initial dose 0.1 mg/kg/day divided bid; maximum of

0.51 mg/kg/day

Metoprolol

PO, 0.2 mg/kg/day divided bid; maximum dose 12

mg/kg/day

Steps in management of CCF:

i. Elimination of underlying cause:
1. Surgery for defects
2. Drugs for hypertension; hyperthyroidism
3. Pacemaker for arrhythmias
ii. Treatment of contributing cause ( fever, anemia, infection)
iii. Control of CCF :
1. Semi upright position (infant chair)

50

2. O2
3. High calorie diet in frequent small feeding
4. Diet:
i.
Low salt diet(<.5 g/day) and fluid restriction in older children;
a. Human breast milk is the ideal low sodium nutritional source.
b. Formula or EBM can be enriched by adding:
1. Medium-chain triglycerides (MCT) oil
2. Microlipid (safflower oil emulsion),
3. Polycose
c. Older children can be managed with no added salt diets
iv. Diuretics:
a. Thiazides no longer popular
b. Frusemide and ethacrynic acid are used
c. Spironolactone used in combination with other diuretics
d. Side effects:
1. Hypokalemia
2. Hypochloremic alkalosis
v. Acute condition:
1. Dopamine for ianotrophic and vasodilator actions (5-10 mcg/kg/min
IV)
2. Amrinone, a phosphodiestrase inhibitor has similar action
vi. Digoxin:
1. Slows HR
2. Increases diuresis
3. Increases contractility
4. Oral or IV; IM not recommended
5. IV dose is 75% of oral dose
6. Dose:
Total dose is divided into 3 doses:
1/2dose initially
1/4dose after8hs
1/4dose after another 8hs
Maintenance dose is divided into 2 equal doses every 12 hs

vii.

Maintenance
g/kg/day

Tdd g/kg

age

20

Premature

30

Newborn

10-12

40-50

Under2ys

8-10

30-40

Over 2ys

Beta -

Blockers:
a. Helps in counteracting adrenergic overstimulation in CCF
b. Given only in compensated CCF with adequate fluid balance and BP
c. Carvedilol and metoprolol used in dilated Cardiomyopathy
d. Propranolol used with some benefits in large Lt to Rt shunts
viii. Carnitine: a cofactor for transport of long chain fatty acids is used for dilated
Cardiomyopathy
ix. Surgery: cardiac transplant when other measures fail.
SYSTEMIC HYPERTENSION:

51
1. Definitions:
1. Normal: systolic/diastolic < 90th percentile; for age and sex on more
than 3 occasions
2. Normal BP:
Age
Systolic
Diastoli
c
0-3 mo
65-85
45-55
1-3 yr
90-105
55-70
6-12 yr
110-135
65-85
>12 yr
110-135
65-85
3. Causes:
1. Primary: idiopathic
2. Secondary
1.
Renal parenchymal disease
1. Glomerulonephritis
2. Pyelonephritis
3. Polycystic kidney
4. Hydronephrosis
5. Haemolytic uremic syndrome
6. SLE
7. Nephrotoxic drugs
2.
Renal arterial disease:
1. Stenosis
2. Thrombosis
3. Cardiovascular: systolic hypertension
1. Coarctation of Aorta
4. Endocrine:
1. Pheochromocytoma
2. Neuroblastoma
3. Cong.adrenal hyperplasia
4. Cushing syndrome
5. Hyperaldosteronism
5. Neurological:
1. Increased intracranial pressure
2. Guillain Barre syndrome
3. Riley-Day-syndrome
6. Drugs:
1. Steroids
2. Contraceptives
7. Miscellaneous:
1. Hypernatremia
2. Stevens-Johnson syndrome
8. Obesity
4. Physical findings:
1. High BP measurement in all limbs in recumbent and erect postures
2. Delayed growth- renal disease
3. Collapsing pulse: AI; PDA
4. Absent femoral or Radio femoral delay: COA
5. Abdominal bruit- renal artery stenosis
6. Renal angle tenderness- UTI
7. High Body mass index- obesklity
5. Lab:
1. Urine analysis

52
2. Urine culture
3. Electrolytes: for hypokalemia
4. BUN
5. Serum creatinine
6. Uric acid level >5.5 mg/dl
7. ECG
8. Echo
9. Lipid profile
10.Thyroid profile
11.Blood glucose levels
6. Specialized studies:
1. Excretory urography
2. Plasma rennin activity
3. Aldosterone level
4. Urinary catecholamine- pheochromocytoma
5. Urinary VMA levels- neuroblastoma
6. Renal sonography and radio nucleotide scan - renal scarring
7. Renal Doppler and angiography
7. Management:
Primary hypertension:
1. Non pharmacologic:
1. Weight reduction
2. Low salt food
3. Aerobic exercise
4. Avoid smoking\avoid oral contraceptives
2. Pharmacologic:
1. Start with thiazide and add other drugs as per response
2. Diuretics: produce hyponatremia, hyperglycemia,
hypercholesterolemia
a. Hydrochlorothiazide: 1 mg/kg/day od ;
b. Furosemide: .
1 to 2 mg/kg/day od or bd
c. Spironolactone:
1 mg/kg/day od or bd
d. Triamterene:
1-2 mg/kg/day bd
3. Adrenergic inhibitors: contraindicated in asthma and diabetes
a. Propranolol :
1-2 mg/kg/day 2 or 3 times
b. Metoprolol:
1-2 mg/kg/day bd
c. Atenolol:
0.5 to 1 mg/day 1-2 doses
4. ACE inhibitors:
leukopenia; cough
a. Captopril:
0.3 to .5 mg /dose 3 doses
b. Enalapril:
0.08 mg/kg/day 1-2 dose
c. Lisinopril:
0.07 mg/kg/day 1 dose
5. Angiotensin receptor blocker: angioedema
a. Losarton:
0 .07 mg/kg/day 1 dose
6. Calcium channel blocker:
headache; flushing; ankle edema
a. Amolodipine:
2.5 5 mg/day 1 dose
b. Extended release nifedipine: 0.25 to 0.5 mg/kg/day 1-2 dose
7. Vasodilators: headache; flushing; SLE; hypertrichosis
a. Hydralazine:
0.75 mg/kg/day 4 doses
b. Minoxidil:
5 mg/day 1-3 doses >12 yrs
2. Secondary hypertension:
1. Surgical correction of COA
2. Treat renal parenchymal disease/ nephrectomy
3. Reconstruction of stenotic renal artery
4. Excision of pheochromocytoma, neuroblastoma etc
8. Hypertensive crisis:

53
1. Rapidly rising BP; neurological manifestation; heart failure; pulmonary
edema
2. Accelerated malignant hypertension: Papilledema; hemorrhage;
exudates
3. Hypertensive encephalopathy: severe headache; altered
consciousness;
4. Treatment:
1. Labetalol .2 to 2 mg/kg/hour IV drip
2. Diazoxide 3-5 mg/kg iv bolus or
3. nitroprusside
1-3 g/kg IV per minute as drip
4. Hydralazine
0.15 mg/kg IV/IM
4. Nifedipine 0.2 to 0.5 mg/kg(up to 10 mg) orally every 4-6
hours
5. Furosemide
1 mg/kg IV

MYOCARDITIS
Overview
1. Major cause of Myocarditis is viral - Coxsackie B
2. Myocarditis is less common: 1 per 100 000
3. Most children will present with nonspecific respiratory or gastrointestinal
complaints
4. Sudden death is a possibility particularly in athletes
5. Many develop dilated cardiomyopathy requiring long term treatment and
cardiac transplant
ETIOLOGY AND EPIDEMIOLOGY
1. Viral myocarditis is typically a sporadic, but occasionally an epidemic illness.
2. Age:
1. Infancy: occurs as an acute, fulminant disease;
2. Toddlers and young children: acute, but less fulminant myopericarditis;
3. Older children and adolescents: asymptomatic ; a precursor to
idiopathic dilated cardiomyopathy.
3. Common causative agents:
1. Adenovirus,
2. Coxsackievirus B,
3. Other enteroviruses
4. Less common causes:
1. Protozoan: Chagas' disease: Trypanosoma cruzi,
2. Bacteria: Diphtheritic myocarditis
3. Rickettsia: Rocky mountain spotted fever
4. Fungi: Actinomycosis
5. Protozoa: toxoplasmosis
6. Parasites: trichinosis; Hydatid cysts
7. Immune-mediated: rheumatic fever and Kawasaki
8. Collagen vascular diseases: SLE
9. Toxic myocarditis: drug ingestion (anthracyclines), diphtheria exotoxin,
anoxic agents
PATHOPHYSIOLOGY
1. T cells mediate ongoing injury to the myocardium by further stimulating
cytokine and other mediators release;

54
2. May also become a chronic process dilated cardiomyopathy
Clinical features
1. Presentation varies with age;
2. Asymptomatic to fulminant patterns
3. Acute: Malignant form with sudden onset with anorexia, vomiting, lethargy,
and, circulatory shock.
4. Subacute:
1. Gradual onset of cardiac symptoms
2. History of upper respiratory tract infection or gastroenteritis
3. Have postinfectious or autoimmune component.
CLINICAL MANIFESTATIONS
Neonate
1. Fever,
2. Severe heart failure,
3. Respiratory distress,
4. Cyanosis,
5. Distant heart sounds,
6. Weak pulses,
7. Tachycardia out of proportion to the fever,
8. Mitral insufficiency caused by dilatation of the valve annulus,
9. Gallop rhythm, acidosis, and shock.
10.Evidence of viral hepatitis, aseptic meningitis, and an associated rash may
be present.
11. death may occur within 17 days
12.X ray Chest: enlarged heart and pulmonary edema;
13.ECG:
1. sinus tachycardia, reduced QRS complex voltage,
2. Arrhythmias may be the first clinical manifestation
Older patient
1. Acute or gradual congestive heart failure;
2. A sudden onset of ventricular arrhythmias.
3. Signs of cardiac dysfunction:
1. Respiratory distress
2. S3 or S4 gallop
3. Mitral or tricuspid incompetent murmurs
4. tachycardia out of proportion to fever
5. Hepetomegaly
6. Chest pain
Diagnosis
1. Heart failure or arrhythmia preceded by a prodrome of flulike symptoms
2. The gold standard for diagnosis is endomyocardial biopsy (more risk)
3. ECG: Sinus tachycardia with low-voltage QRS complexes and inverted T
waves; premature ventricular or atrial beats, arrhythmias, chamber
hypertrophy etc
4. X-Ray: cardiomegaly and pulmonary venous congestion
5. CBC, ESR, and CRP for markers of inflammation.
6. Blood culture, PCR of tracheal aspirates, specific viral titers in blood, nasal
culture, and rectal culture particularly for isolation of enteroviruses
7. Cardiac troponin T is increased

55
8. Echo: left ventricular or biventricular dysfunction, dilation, and wall motion
abnormalities; mitral and tricuspid valve regurgitation and atrial enlargement
9. Cardiac magnetic resonance imaging (CMRI) identifies areas of myocardial
inflammation g
10.Biopsy is performed during cardiac catheterization and can also be used to
detect other causes of cardiomyopathy
DIFFERENTIAL DIAGNOSIS
1. Carnitine deficiency:
Carnitine is an amino acid that is required for the transport of long-chain fatty
acids into the mitochondria; helps to produce energy from fat; fasting or viral
infections is risk for heart failure and sudden death.
2. Hereditary mitochondrial defects:
Dysfunction of the mitochondrial respiratory chain; essential final common
pathway for aerobic metabolism; Tissues with a high energy demand (eg,
brain, nerves, retina, skeletal and cardiac muscle) are particularly affected.
3. Idiopathic dilated cardiomyopathy: genetic defect cardiomyopathy; heart
failure may occur suddenly
4. Fibroelastosis of the endocardium: fibroelastic thickening of the endocardium
; primary and secondary; CCF during resp.Infection
5. Anomalous origin of the left coronary artery from pul.artery (ALCAPA) : Bland
White Garland syndrome; left coronary artery empties into the pulmonary
artery, a condition known as the myocardial steal syndrome; may die of
heart failure within the first year of life if not treated
Treatment
1. Manage heart failure and arrhythmias
2. Inotropes, diuretics
3. Digoxin may enhance the proinflammatory mediators
4. Mechanical ventilation to reduce workload.
5. Extracorporeal membrane oxygenation (ECMO)
6. Antimicrobial and antiviral therapy
7. Intravenous immunoglobulin
8. Myocarditis caused by autoimmune disease will most likely benefit from
immunosuppressive therapy.
9. Heart transplant
10.Bed rest
Prognosis
1. Complete recovery, 66% in adolescents
2. Progression to DCM, 12% to 40%
3. Death- newborns 75% mortality
Pericarditis
Etiology:
1. Viral infection
2. Acute rheumatic fever
3. Bacterial infection
1. S. aureus, Streptococcus pneumoniae, Haemophilus influenzae,
Neisseria meningitidis, and streptococci.
2. 4. Tuberculosis is an occasional cause of constrictive pericarditis, with
an insidious onset.

56
4. Heart surgery
5. Collagen disease
6. Oncologic therapy, including radiation.
7. Uremia
Pathopysiology
1. Accumulation of fluid in the pericardial space.
2. May be serous, fibrinous, purulent, or hemorrhagic.
3. 1015 ml of fluid is normal
4. Cardiac tamponade occurs when fluid reaches a level that compromises
cardiac function.
5. Results in severe cardiac compression. Inhibition of ventricular filling during
diastole, elevated systemic and pulmonary venous pressure, compromised
cardiac output and shock occur
CLINICAL MANIFESTATIONS.
1. Sharp, stabbing sensation over the precordium. left shoulder and back;
exaggerated by lying supine and relieved by sitting, leaning forward.
2. the pain is a referred pain from diaphragmatic and pleural irritation.
3. Cough, dyspnea, abdominal pain, vomiting, and fever may also occur.
Signs
1. Friction rub
2. Muffled heart sounds
3. Narrow pulses,
4. Tachycardia,
5. Neck vein distention,
6. Increased pulsus paradoxus: decrease in systolic pressure during inspiration;
>20 mm in cardiac tamponade
7. Hepatomegaly
DIAGNOSIS.
1. Ecg:
1. Low voltage of the QRS complexes
2. Mild elevation of ST segments
3. Generalized t-wave inversion
4. Variable QRS complex amplitude
2. X-ray chest:
1. Water bottle configuration
2. The lung fields are clear
3. Constrictive pericarditis: the heart is small and pericardial
calcification may be present.
Echocardiogram
1. Echo-free space between the epicardium and pericardium.
2. Posterior effusion is recorded behind the left ventricular epicardium
3. Anterior effusion will be recorded between the chest wall and the anterior
right ventricular wall
4. Flattening of septal motion and collapse of right ventricular outflow during
diastole are signs of pericardial tamponade.
Constrictive Pericarditis
1. Constriction occurs months or years after the initial pericarditis
2. Hepatomegaly and ascites may be out of proportion

57
3. Neck vein distention, narrow pulses, quiet precordium, distant heart sounds,
a faint pericardial friction rub, and increased pulsus paradoxus
4. Calcification of the pericardium
Managemnet
1. Pericardiocentesis to identify the cause of the pericarditis
2. Pericardial fluid studies: cell counts; gram and acid-fast stains; and viral,
bacterial, and fungal cultures.
3. For cardiac tamponade, urgent decompression by surgical drainage
4. Purulent pericarditis: urgent surgical drainage followed by iv antibiotic
therapy for 4 to 6 weeks.
5. There is no specific treatment for viral pericarditis.
6. Treatment of basic disease itself (e.g., uremia, collagen disease).
7. Salicylates are given for precordial pain and nonbacterial or rheumatic
pericarditis.
8. Corticosteroid for rheumatic carditis or postpericardietomy syndrome.
9. Digitalis is contraindicated in cardiac tamponade because it blocks
tachycardia, a compensatory response to impaired venous return
Infective Endocarditis (IE):
1. Incidence: 1 per 1000 admissions
2. Predisposition:
Damaged endothelium due to jet effectsterile thrombus bacteraemia
infected vegetations
3. Common Defects:
a. Morec in TOF, VSD, PA, MVP, MR ; Rare in Secundum ASD
b. Rh. Heart disease
c. Prosthetic heart valve or prosthetic material in the heart
d. Mitral valve prolapse (MVP) with mitral regurgitation (MR)
e. Hypertrophic obstructive cardiomyopathycardiomyopathy
4. Causes for Bacteraemia:
a. abscess, osteomyelitis, pyelonephritis
b. dental procedures,
c. Chewing with diseased teeth or gums
5. Pathology:
Vegetations occur either around the defect or on the opposite surface of the
defect where endothelial damage is established by the jet effect of the
defect.
6. Microbiology:
i. Streptococcus viridians in 60%
ii. HACEK organisms: Haemophilus, Actinobacilus, Cardiobacterium,
Eikenella, Kingella-17% to 30%
iii.
-Hemolytic streptococci (S. viridans): in dental procedures or in
those with carious teeth
iv.
Enterococci: after genitourinary or gastrointestinal surgery or
instrumentation.
v. Staphylococci : postoperative endocarditis, indwelling vascular
catheters, prosthetic material, and prosthetic valve and IV drug users
vi.
Fungal endocarditis (which has a poor prognosis) may occur in sick
neonates,

58

vii.

Culture negative IE in Pt treated with antibiotics

7. Clinical:
a. History of:
i. Underlying Heart disease
ii. Tooth ache or dental procedure
iii. Open heart surgery in infants
iv. Prolonged fever
b. Physical examination:
i. Increasing or newly appearing murmur-100%
ii. Fever-80%
iii. Splenomegaly-70%
iv. Skin manifestations-50%
1. Petechiae on skin and conjunctivae
2. Oslers node: tender pea sized red nodes at ends of
fingers and toes
3. Janeways lesion: haemorrhage on palm and sole
4. Splinter haemorrhage: linear and beneath the nails
v. Thromboembolism: Pulmonary or systemic
vi. Seizures and hemiparesis
vii. Hematuria
viii. Roths spots: retinal haemorrhage near optic disc
ix. Clubbing
x. CCF
xi. NB: nonspecific; Tachypnea, apnoea etc
8. Lab:
a. Blood culture positive in 90%
b. Leukocytosis
c. Anemia
d. Increased ESR
e. Microscopic Hematuria
9. Echo:
a. Oscillating intra cardiac mass
b. Abscesses
10.
Diagnosis: Modified Duke Criteria:
a. Major:
i. Positive blood culture of any of the following:
1. S.viridons or
2. S.bovis or
3. HACEK or
4. S.aureus
ii. 2 Positive blood culture out of 4 at 12 hour intervals of a
possible microbe
iii. Single culture of Coxiella burnetti
iv. Histologic evidence of abscess/ vegetation
v. Positive Echo
b. Minor:
i. Predisposing heart condition
ii. Fever
iii. Thromboembolism
iv. AGN or Oslers node or Roths spot
v. Blood culture excluding those of Major criteria
11.Diagnosis:
a. Definitive:
i. Pathologic:
1. blood culture or

59
2. histological evidence of intra cardiac abscess or
vegetations
ii. Clinical:
1. 2 major and 1 minor or
2. 1 major and 3 minor or
3. 5 minor
12.
Management:
a. Crystalline Penicillin is still a very effective antibiotic for streptococcal
and enterococcal endocarditis
b. For those allergic to penicillin/cephalosporines, vancomycin is the best
alternative
c. Prosthetic valve endocarditis is difficult to treat, carries high mortality.
Rifampin should be added to antibiotic regimen and duration of
treatment should be longer
d. For culture negative endocarditis treatment should start with
vancomycin plus gentamicin
13.Prognosis: 80-90% recovery Poor outcome in fungal IE
14.
Prevention:
a. Oral hygiene
b. Prophylaxis against s.viridans for dental, oral, RS, oesophageal
procedures
c. Against Enterococcus fecalis for GIT, GUT procedures.
d. Amoxycillin oral or parenteral 1-2 doses
e. Prophylaxis against Rheumatic fever and clindamycin for procedure
prophylaxis
15.Risk stratification for IE
Highest risk
Moderate risk
Prosthetic heart valves
Residual lesion after valve repair
Complex cyanotic CHD
Acquired valvular heart disease (RHD)
Previous episodes of IE
16.Prophylaxis for various procedures:
Prophylactic Regimens for Dental, Oral, Respiratory Tract, or Esophageal
Procedures
Situation

Agents[*]

Regimen[+]

Standard general
prophylaxis

Amoxicillin

50 mg/kg orally 1 hr before procedure

Unable to take oral

medication

Ampicillin

50 mg/kg IM or IV within 30 min before

procedure

Allergic to penicillin

Clindamycin

20 mg/kg or orally 1 hr before procedure

Cephalexin

50 mg/kg or orally 1 hr before procedure

Azithromicin or
clarithromycin

15 mg/kg or orally 1 hr before procedure

Prophylactic Regimens for Genitourinary, Gastrointestinal (Excluding

Esophageal) Procedures
High-risk patients

Ampicillin plus Ampicillin 50 mg/kg IM or IV plus gentamicin

Gentamicin
1.5 mg/kg within 30 min of starting the
procedure,
6 hr later, ampicillin 25 mg/kg IM/IV or
amoxicillin 25 mg/kg orally

60
High-risk patients allergic
to ampicillin/amoxicillin

Vancomycin
plus
Gentamicin

Vancomycin 20 mg/kg IV over 12 hr plus

gentamicin 1.5 mg/kg IV/IM; complete
injection/infusion within 30 min of starting the
procedure

Moderate-risk patients

Amoxicillin or
Ampicillin

Amoxicillin 50 mg/kg orally 1 hr before

procedure, or ampicillin 50 mg/kg IM/IV within
30 min of starting the procedure

Moderate-risk patients
allergic to
ampicillin/amoxicillin

Vancomycin

Vancomycin 20 mg/kg IV over 12 hr; complete

infusion within 30 min of starting the procedure

Kawasaki Disease: Muco cutaneous lymph node syndrome

1. Epidemiology:
a. Due to abnormality of immune system initiated by the infectious
insult
b. More common in Asians; Male : female= 1.5:1; more during winter
c. Peak in 1-2 years; uncommon in < 3 mo and > 8 yrs
2. Pathology:
a. About 10 days after fever generalized microvasculitis appear; more
predilection for coronary vessels; can involve iliac, femoral, axillary
and renal arteries
b. Coronary artery aneurysm in 15-20%
c. Acute: AV block, pericardial effusion, CHF and valvular lesions can
occur
d. Late: after 40 days, coronary artery thrombosis, post myocardial
infarction fibrosis develop
e. Elevated platelet counts contribute to thrombosis
3. Clinical Manifestation: acute, sub acute and convalescent
Acute (first 10 days):
a. 6 signs
i. Abrupt onset of high fever(104F) persisting for at least 5
days
ii. Conjunctivitis that resolves rapidly
iii. Erythema of mouth,
iv. lip cracking, strawberry tongue; Erythema of palm and soles;
oedema of hands
v. Polymorphic skin eruptions
vi. Cervical lymphadenopathy- unilateral
b. CVS involvement:
i. Tachycardia, gallop
ii. Cardiomegaly
iii. Pericardial effusion
iv. Mitral regurgitation
v. Coronary abnormalities
4. Lab:
i. Leukocytosis
ii. Acute phase reactants
iii. Thrombocytosis
iv. Pyuria due to urethritis
v. Increased cardiac troponin -1 (myocardial damage)
vi. Low HDL
5. ECHO:
i. perivascular brightness( coronary arteritis)

61
ii. coronary artery aneurysm
iii. LV dysfunction
iv. Pericardial effusion
6. Subacute: 11 25 days
a. Desquamation of tips of fingers and toes
b. Coronary artery aneurysm
c. Thrombocytosis
7. Convalescent:
a. Elevated ESR
b. Thrombocytosis
c. Deep transverse groove (Beaus line) in finger and toe nails
8. Diagnosis:
a. Fever for 5 days or more with any 4 out 6 signs
b. Incomplete Kawasaki if some criteria are missing
9. DD:
a. Measles
b. Group A b-haemolytic streptococcal infection
c. Kawasaki: irritability more; acute phase reactants continue even
after 7 days
10. Management:
a. High dose IV IG 2 G/kg as 10-12 hr single infusion
b. Aspirin 80-100 mg/kg / day for 3-5 days followed by 3-5 mg/kg/day
OD
c. Steroids if IV IG 2-3 doses fail
11. Natural History:
a. Coronary artery aneurysm in 15-25 %
b. Myocardial infarction in 70% in first year
12. Follow up:
a. Aspirin prophylaxis for aneurysm
b. Exercise restriction
c. Regular cardiac evaluation for coronary aneurysm