Congenital heart

disease

Ms.Kusum, MSc.Nursing,2nd year

Congenital heart
disease
congenital cardiac malformations
resulting
from defective embryonic
development
without obvious
cause.
Between 3-8 weeks gestation all the
fetal
heart structures are formed
organogenesis

Incidence

8-10/1000 live births in india

approximately 2000/ year
Incidence is more in :a-Premature
b-abortions
c-still births
Incidence increased for siblings.
2-6%,10-12%,25%

Etiology
Multifactorial inheritance pattern mostly

Chromosomal abnormality (5-10%).

-Trisomy 21 (50%) > A-V canal,VSD,ASD, others.

-Trisomy 18 (80%)> VSD,ASD,others.
-Trisomy 13 (40%)> VSD,ASD,PDA,others.
-Turner syndrome (xo)>Bicuspid aortic valve and co-ao
-others.

Adverse maternal conditions

(enviromential)
-Maternal infections >Rubella:PDA,PS

-Maternal diseases> VSD,ASD

left septal hypertrophy
-Fetal alcohol syndrome> VSD,ASD,CO-AO.
-Advance maternal age.

 Syndrome complexes

-VACTREL syndrome

Vertebral,Anorectal,Cardiac(VSD,TOF and
others),tracheal,Renal,Oesophageal and Limb
abnormalities.

-CHARGE syndrome

Coloboma(congenital malformation of the eye causing

defects in lens, iris or retina),Heart(VSD,TOF,A-V canal),

Atresia(absence or abnormal narrowing of an opening
or passage in the body),Retardation,Gential,Ear
abnormalties.
-Kartagener syndrome Dextrocardia

Majority of cases of the

congenital heart
diseases are unknown
cause

Classification

C.H.D with little or no cyanosis acyanotic

a-With Lt. ventricular enlargement
ex.- PDA=8% ,CO-Ao= 5%
- aortic stenosis =7%
b-With Rt. Ventricular enlargement
ex.-ASD=10% ,PS=6%,MS
c-With both ventricular enlargement
ex.- VSD= 25%

Common acyanotic
lesions
Ventricular septal defects
Atrial septal defects
Atrio-ventricular septal defects
Patent ductus arteriosus
Truncus arteriosus
Aortic stenosis
Mitral stenosis/incompetence
Coarctation of aorta
Tricuspid regurgitation

C.H.D with cyanosis mainly Rt. Lt.

shunt
a-With decrease pulmonary vascularity
ex.-TOF=5% , Tricuspid Artesia=1-3%
- PS with or without VSD=5%
b-With increase pulmonary vascularity
ex.-TGA =5% ,Truncus arteriosus=2%
-TAPVR=1%

Common Cyanotic Lesions

Decreased flow
1. Tetralogy of Fallot
2. Tricuspid Atresia
3. Severe Pulmonic Stenosis
4. Ebsteins anamoly
Increased Flow
5. Transposition of great vessles
6. VSD with pulmonary atresia

Cont-Common Lesions
producing cyanosis
7.
8.
9.

Truncus Arteriosus
Hypoplastic left heart
Single ventricle

10. TAPVR with

infradiaphragmatic

obstruction

Prevalence

Cyanotic: 22%
Acyanotic: 68%

VSD
ASD
PDA
TOF
PS
AS

25%
6%
6%
5%
5%
5%

Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7
Nelsons Textbook of pediatrics; 17 ed.

Diagnosis
Early diagnosis of C.H.D mean better
results.
40% of C.H.D diagnosed at 1st w of life.
50-60 % diagnosed at 1st two months .
Others are usually later during routine
medical examination.
Diagnosis depend on good clinical history
+ good medical examination and
investigations.

History
Age of the patient.
Ask for
-Feeding difficulties

D.D
Sepsis
Neonate

Metabolic disorders
-Vomiting
Hypothermia
-Lethargy
-Increased perspiration
IVH
-Rapid respiration
Infant
Others
-Hypoactivity
-F.T.T
All may be manifestation of congestive heart
failure.

Older child

-History of dysnea on exertion.

-Shortness of breathing.
-Orthopnea.
-Lower limbs swelling
-Palpitation.
-Convulsion.
-All may be manifest of congestive heart failure.

Physical examination

Inspection look for:

-Cyanosis
- clubbing
-Tachypnea
-Prominence of the precordium (cardiomegaly,Rt.heart enlargement )
-Jugular veins engorgement older children
-Any associated defects or findings (down syndrome, edward
syndromeetc)
- Focal neurological lesion.

Palpation
-Pulses (rate, rythem,volume,peripherial
pulses ,brachio-femoral delay)
-Cardiac impulses.
-Thrill.
-Hyper dynamic precordium.
-Hepato-splenomegaly
-Sacral edema(neonate, and infancy)
,Lower
limb edema in older children

Auscultation
a-First heart sound (A-V valves closure)

Best heard at the Lt. lower sternal border or apex

b-Second heart sound (semilunar valve closure)
Best heard on the 1st and 2nd I.C.S , normally
there

is normal

splitting of the 2nd heart sound ,

-Single
Aortic atresia,Pulmonary Artesia
-Fixed splitting Systolic
ASD,PS,Rt.B.B.B
c-Murmurs
Diastolic
Continous

Blood pressure

Wide pulse pressure

-Aortic insufficiency
-A-V communication
-PDA
Low blood pressure(H.F, pericardial
tamponade,cardiomyopathy).
Difference in BP between upper and lower
extremities
Co-ao.

If we suspect C.H.D
Investigation

CBC---- polycythemia, anemia.etc

CXR----heart size and shape
ECG---HR,axis www.heartaxis.com
,rythm
LVH,RVH,BVH,BBB.
Echocardiography
MRI
Cardiac catheterization

Cyanosis: is it a
cardiac cause or lung
cause
Hyperoxia test
Neonates with cyanotic congenital
heart disease usually do not have
significantly raised arterial Pao2
during administration of 100%
oxygen.

Ventricular Defect

Small VSD
Asymptomatic
A loud, harsh, or
blowing systolic
murmur.

Large VSD
dyspnea, feeding
difficulties, poor
growth, profuse
perspiration,
recurrent pulmonary
infections, and
cardiac failure in
early infancy.

80%

Ventricular Septal Defect

(VSD)
Small VSDs, the chest radiograph is usually normal

Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields
(pulmonary hypertension or an associated pulmonic stenosis), gross
cardiomegaly with prominence of both ventricles, the left atrium.

Ventricular Septal
defects

3050% of small defects close spontaneously,

most frequently during the 1st 2 yr of life.
Small muscular VSDs are more likely to close (up
to 80%) than membranous VSDs are (up to 35%).
infants with large defects have repeated episodes
of respiratory infection and heart failure despite
optimal medical management.
Surgical repair prior to development of an
irreversible increase in pulmonary vascular
resistance (usually prior to the patient's second
birthday).

Atrial Septal Defects:

secundum

Most common form of

ASD (fossa ovalis)
In large defects, a
considerable shunt of
oxygenated blood
flows from the left to
the right atrium.
Mostly asymptomatic
The 2nd heart sound
is characteristically
widely split and fixed.

Secundum

Atrial Septal
Defects:primum

Situated in the lower portion

of the atrial septum and
overlies the mitral and
tricuspid valves. In most
instances, a cleft in the
anterior leaflet of the mitral
valve is also noted.
Combination of a left-to-right
shunt across the atrial defect
and mitral insufficiency
C/F similar to that of an
ostium secundum ASD

Atrial Septal Defect

Atrial Septal Defect

Enlargement of the
right ventricle
Enlargement of
atrium
Large pulmonary
artery
increased
pulmonary
vascularity is.

Atrial Septal Defect

Three major types

Ostium secundum
most common
In the middle of the septum in the region of the
foramen ovale

Ostium primum
Low position
Form of AV septal defect

Sinus venosus
Least common
Positioed high in the atrial septum
Frequently associated with PAPVR

Atrial Septal Defects

Secundum ASDs are well tolerated during

childhood.
Antibiotic prophylaxis for isolated
secundum ASDs is not recommended.
Surgery or transcatheter device closure is
advised for all symptomatic patients and
also for asymptomatic patients with a
Qp:Qs ratio of at least 2:1.
Ostium primum defects are approached
surgically

Patent Ductus
Arteriosus

Small defect no
symptoms.
Large defect:

Wide pulse pressure

Enlarged heart
Thrill in L second IS
Continuous murmur
X-ray: prominent
pulmonary artery
with increased
vascular markings.

Patent Ductus
Arteriosus
Treatment consists of surgical correction
when the PDA is large except in patients
with pulmonary vascular obstructive disease
Transcatheter closure of small defects has
become standard therapy
In preterm infants indomethacin is used (8090% success in infants > 1200 grams)

Primary Pulmonary
Hypertension

Prominent
pulmonary artery.
Prominent right
ventricle
Prominent
vascularity in the
hilar areas
Decreased
vascualr marking
in the periphery.
No treatment

Cardiac disease with

normal/decreased vasculature

Tetralogy of Fallot
Pulmonary atresia
Tricuspid atresia
Endocardial fibroelastosis
Aberrant left coronary artery
Cystic medial necrosis
IODM septal hypertrophy

Tetralogy of Fallot

Ventricular septal
defect
Pulmonic stenosis
Overriding aorta
Right ventricular
hypertrophy
Cyanotic

Tetralogy of Fallot
Cyanosis, especially in the adult, is the result of a small number
of cardiac malformations well determined. Oneis much more
frequent than the others. This malformation consists of a true
:anatomopathologic type represented by the following tetralogy
;Stenosis of the pulmonary artery) 1 (
; Interventricular communication) 2 (
Deviation of the origin of the aorta to the right; and) 3(
Hypertrophy, almost always concentric in type, of the right ) 4(
. ventricle
Failure of obliteration of the foramen ovale may occasionally be
.added in a wholly accessory manner

Tetralogy of Fallot

Tetralogy of Fallot
Most common cyanotic lesion (7 to
10% of all CHD)
Typical features
Cyanosis after the neonatal period.1
Hypoxemic spells during infancy.2
Right-sided aortic arch in 25% of all.3
patients

Tetralogy of Fallot

Tetralogy of Fallot
Repair may be staged (modified
BT shunt) or complete

Treatment of C.H.D

This is depend on the type of the C.H.D.

No treatment (observation+reassurance)
Medical
treatment(antifailure,antiarythmaic..etc).
Surgical treatment (palliative or curative).
Cardiac transplant or lung heart transplant.

General measures-1

Special positions. (semisiting ,knee chest position (

O2 (most patients need O2 and other need little O2).

IVF(again depend on type of CHD , some need IVF as PDA an

, OTHERS need IVF when ever there are pulmonary congesti
volume overload).

Salt restriction.

Exercise restriction.

Rx of anemia.

Rx of polycythemia. PCV>65
Avoidances of dehydration mainly polycythemic
patients.
Avoidances of high altitude.
Avoidance of contraceptive
thrombosis+hypertension.
Correction of acidosis.
Correction of electrolyte disturbances .
Careful monitoring during surgery.

Rx of congestive-2
heart failure
Digoxin

Digitalization 0.04mg/kg
Maintenance 0.01mg/kg
Loop diuretics frusemide 1-2 mg/kg/day.
Potassium sparing diuretics spironlactone
After load reducing agents
eg. Captopril 0.5-6mg /kg/24 hours.
Positive intropic agents .dopamine and
dobutamine

Rx of cardiac-3
arrhythmias

Digoxin (be aware of toxicity nausia ,vomiting,AV block, K, ca).

Quinidine( hemolytic anemia.SLE,perodic paralysis)
Procainamide(agranulocytosis,+ve coombs hemolytic anemia )
Lidocaine (confusion, convulsion,respiratory failure,AV block).
Propranolol(bradycardia,bronchspasm,hypotensionetc).
Verapamil(bradycardia,PR interval prolongation,CHF).
Adenosine(chest pain,flushing,bronchospasm,bradycardia )

Rx of the cyanotic-4
spells

Try to calm the patient .

Knee chest position,
O2
Propranolol IV.
Morphine s.c
NaHCO3 iv
Increase IV fluid.

Prophylaxis against infective-5

endocarditis

By oral or parental antibiotics.

Given in case of dental
procedures, urinary and GIT .
Instrumental
proctoscopy,cystescopy.
Now a days lot of controversy
about who should take the
prophylactic antibiotics.

prostglandine PGE2,
AND indomethacine for
selective cases of CHD

Preventive measures

Avoid pregnancy above age of 38 year.

Avoid smoking.
Avoid alcohol.
Avoid exposure to radiation.
Avoid drugs intake before medical
consultation.
Vaccination of all childbearing women
for rubella.

Innocent murmur
It is functional,normal,insignificent.
Patient is asymptomatic.
Murmur heard on routine cardiac
examination without pathological
causes.
Main age between 3-7 years.
Incidence : up to 30% especially child
with fever,infections,anxiety,and with
cardiac output, the incidence is
decreased with age.

Characteristics of the
innocent murmur
Mild murmur usually grade one.
Brief in duration.
Changing with position.
No radiation.
It is systolic,musical,shortand
ejectional.
Best heard over Lt. lower sternal
and midsternal border.