adiuvante Dei gratia doctorum factionis
CARDIO:
CONGENITAL HEART DISEASES
CONGENITAL HEART DISEASE
NOBELANG INTROXD
Initially congenital heart disease will be tolerable but later it
will end up with problems.
Why are we studying these congenital heart diseases?
Because later, the less complex congenital heart
disease will have complications.
If you would notice the normal circulation:
from SVC and IVC right atrium tricuspid valve
right ventricle pulmonary valve pulmonary
arteries lungs pulmonary veins left atrium
mitral valve left ventricle aortic valve
aorta
If we would have a patient with Atrial Septal defect, the
circulation is:
right atrium tricuspid valve right ventricle
pulmonic valve pulmonary arteries lungs
pulmonary veins left atrium some of the blood
goes back to right atrium due to a hole in the atrial
septum (Initially) resulting in more blood volume in
the right side of the heart, even in the pulmonary
circuit (there is VOLUME OVERLOAD) which may later
lead to complications caused by volume overload
such as pulmonary hypertension
COMPLICATIONS OF CHD:
1. PULMONARY ARTERIAL HYPERTENSION
2. EISENMENGER SYNDROME
3. ERYTHROCYTOSIS
4. INFECTIVE ENDOCARDITIS
1. PULMONARY HYPERTENSION
For unknown reason, with that increase in pulmonary blood
flow, you will end up having fibrotic changes within the
pulmonary parenchyma which will not allow the normal blood
flow coming from the pulmonary arteries to the pulmonary
arterial circuit, and if you have an abnormal blood flow,
pressure will increase within the pulmonary arteries
PULMONARY ARTERIAL HYPERTENSION (PAH)
hindi kasi makalusot yung dugo, dapat dadaan lang yung dugo
sa pulmonary vessels, e kung di nakakalusot yung dugo, anong
mangyayari sa volume and pressure within the pulmonary
artey?within the right ventricle? Within the right atrium?
The first thing that will happen is we end up having PAH
So when there is PAH, blood pressure is higher as compared to
right ventricular pressure resulting to back flow of blood from
pulmonary arteries to right ventricle then to right atrium (which
has lesser pressure)
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Page 1 of 12
2014-2015
DR.
BARTOLOME
2. EISENMENGER SYNDROME
If there is Atrial Septal defect, if the pressure in right atrium is
HIGHER than pressure in left atrium (usually, left atrium has
higher pressure as compared to right side) blood will flow from
Right atrium to Left atrium, and that blood (unoxygenated) will
go to the left ventricle then to the aorta then to systemic
circulation CYANOSIS
And this condition is known as EISENMENGER SYNDROME
*EISENMENGER SYNDROME
o the process in which a left to right shunt caused by
a congenital heart defect in the fetal heart causes
increased flow through the pulmonary vasculature,
causing pulmonary hypertension, which in turn causes
increased pressures in the right side of the heart and
reversal of the shunt into a right-to-left shunt. (may
reversal of shunt!)
the principal determinant of clinical manifestation for the
feasibility of surgical repair is if the patient has an
uncomplicated congenital heart disease (Ex. ASD without PAH)
Imagine, the lungs of the patient is destroyed because of the
changes, but you can patch the hole in the intraatrial septum
using a pericadium patch using a device (yung ginawa kay kuya
Kim, kaya nonexistent na yung ASD nya)
BUT if you do this surgery to a patient with PAH, USELESS LANG
ang closure of the intraatrial septum kasi nga fibrotic na yung
pulmonary vessels, mataas parin ang pressure sa pulmonary
arteries, thus there will still be right sided heart failure UNLESS
you transplant new lungs to the patient.
In the near future baka pwede na magpagawa ng lungs thru
cloning (haha) then pwede na palitan yung baga. Magpagawa
ka ng clone mo para hindi ka agad mamatay, papalit ka lang ng
papalit ng defective organs mo. Unethical ba yun? pinapahaba
mo lalo yung buhay? (harhar) etc. about cloning
3. ERYTHROCYTOSIS
How do you diagnose Pulmonary Hypertension?
o There is pressure within pulmonary artery, thats why
you call it PAH, and if that pressure is HIGH, what
will happen to that pressure? It will go SOMEWHERE
ELSE right ventricle right atrium right sided
heart failure manifestation (neck vein engorgement,
ascites, generalized edema) and erythrocytosis
Why Erythrocytosis?
o In ASD with pulmonary hypertension (Eisenmenger
syndrome), wherein the right atrial pressure is higher
than left atrial pressure (so blood flows from right to
left atrium), UNOXYGENATED blood is being pumped
out of arterial circuit (dahil may reversal of shunt),
there will be hypoxemic blood in the left ventricle and
in the arterial circuit which be sensed as HYPOXIA by
the body. When the body senses hypoxia, it will
stimulate the kidneys to produce erythropoeitin
inc. RBC production ERYTHROCYTOSIS.
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 o Then pag sobra yung RBCs, patient is more prone to
develop thrombosis (thats why you do phlebotomy
in patients with very high Hgb and Hct)
On the other hand, in pateints with Tetralogy of Fallot, syempre
hypoxemic na yung patient, you give aspirin (antithrombotic),
but they have paradoxical bleeding (for unknown reasons),
they have a tendency to bleed, but we still give aspirin for the
viscosity of the patients blood kaya mejo mahirap din yung pag
balance ng pag bigay ng aspirin in a patient with hypoxia
secondary to congenital heart disease.
4. INFECTIVE ENDOCARDITIS
Patients with CHD are prone to develop INFECTIVE
ENDOCARDITIS.
INFECTIVE ENDOCARDITIS
o Infection of the endocardium
o Microbacterium is inside the endocardium, can you
imagine, for EVERY cardiac contraction there would
be bacteria released in the circulation bacteremia
o if it goes to the brain brain abscess
o if it goes to the eyes blindness
o if it goes to the heart coronary artery infection
So patients with Congenital Heart Disease should have
Infective Endocarditis Prophylaxis (IE prophylaxis) especially
those undergoing dental procedures, GUT and GIT diagnostic
procedures. Give antibiotic therapy to ALL patients with
valvular heart disease prior to these procedures EXCEPT FOR:
o ASD, ostium Secundum type
o Pure mitral valve prolapse
For the rest, or If youre not sure, just give your IE prophylaxis.
For dental procedures: give oral Amoxicillin (3 grams prior to
procedure then 1.5 grams after the procedure
So pag may murmur ang patient, magbigay nalang ng IE
prophylaxis
Other measures:
o clean teeth
o dental abscesses must be removed
Does brushing of teeth produce bacteremia?
o Common oral hygiene, such as brushing teeth or
flossing, can cause transient but harmless bacteremia.
Some patients with prosthetic heart valves however
need antibiotic prophylaxis for dental surgery because
bacteremia might lead to endocarditis (Wikipedia) so
YES.
Infective Endocarditis
o While anyone can contract endocarditis, the infection
usually develops in individuals with underlying
structural cardiac defects. It can occur whenever
these persons develop bacteremia with the organisms
likely to cause endocarditis. Bacteremia may occur
spontaneously (i.e., caused by organisms introduced
through food chewing or tooth brushing), or it may
develop as a complication of a focal infection, such as
a periodontal or periapical infection, a urinary tract
infection, pneumonia or cellulitis.
o Selected surgical and dental procedures and
instrumentations, especially those involving mucosal
surfaces or contaminated tissue, can cause a transient
bacteremia that rarely persists for more than a few
minutes.
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o For endocarditis to develop, two independent events
are normally required:
an area of endothelium must be damaged,
and a bacteremia caused by adherent
organisms must occur. (AHA)
ASD- MOST COMMON CHD in ADULTS
VSD- MOST COMMON CHD for ALL AGES
EPIDEMIOLOGY
1% of all live births
4% of offspring of women with congenital heart disease
Generally the result of aberrant embryonic development of a
normal structure or failure of such a structure to progress
beyond an early stage of embryonic or fetal development
Complex multifactorial genetic and environmental causes
Chromosomal aberrations and mutations of single genes
account for 10% of all cardiac malformations
CLASSIFICATION OF CHD
A. ACYANOTIC with Left-to-Right shunt
I. Atrial level shunt
a. Atrial septal defect (ASD)
b. Atrial septal defect with mitral stenosis (Lutembachers
syndrome)
c. Partial anomalous pulmonary venous connection
II. Ventricular level shunt
a. Ventricular septal defect (VSD)
b. VSD with aortic regurgitation
c. VSD with left ventricular to right atrial shunt
III. Aortic root to right heart shunt
a. Ruptured sinus of Valsalva aneurysm
b. Coronary arteriovenous fistula
c. Anomalous origin of the left coronary artery from the
pulmonary trunk
IV. Aortopulmonary level shunt
a. Aortopulmonary window
b. Patent ductus arteriosus
V. Multiple level shunts
a. Complete common atrioventricular canal
b. VSD with ASD
c. VSD with PDA
B. ACYANOTIC without a Shunt
Here, the problem is MALDEVELOPMENT of certain structures.
For example:
a fibrotic lesion in the pulmonary veins at birth
Pulmonary vein stenosis (also a CHD)
a deformed mitral valve after birth mitral stenosis
problem secondary to CHD
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I. Left heart malformations right chamber of the heart will decrease increased right
a. Congenital obstruction to left atrial inflow ventricular diastolic filling pressure blood in the right
1. Pulmonary vein stenosis atrium CANNOT easily go to the left ventricle
2. Mitral stenosis In patients with ASD, the pressure now will go to the right
3. Cor triatriatum atrium blood flows form LA to RA, blood can now easily
pass through the intra atrial septum there will be
b. Mitral regurgitation adequate relaxation of the left ventricle, Atrial septal
1. Atrioventricular septal (endocardial cushion) blood flow can be regained
2. Congenitally corrected transposition of the great On the other hand, if there is a left ventricular
arteries dysfunction can happen in ASD, VSD, and PDA)
3. Anomalous origin of left coronary artery from the 3. Relative impedance in the pulmonary and systemic
pulmonary trunk circulations
c. Aortic stenosis pulmonary circulation:
d. Aortic valve regurgitation o if there is fibrotic changes within pulmonary
e. Coarctation of the aorta parenchyma due to increased blood flow over
time inhibition of blood flow from pulmonary
II. Right heart malformations artery to lungs PAH
a. Acyanotic Ebsteins anomaly of the tricuspid valve o If there is NO pulmonary blood flow problem, no
b. Pulmonic stenosis fibrotic changes, blood can easily flow
c. Congenital pulmonary valve regurgitation Systemic circulation:
d. Idiopathic dilation of the pulmonary trunk o If there is INCREASED. SVR INCREASED.
afterload INCREASED PRESSURE in Left
C. CYANOTIC with a shunt ventricle)
Left-to-right shunt causes diastolic overloading of the RV
I. Increased pulmonary blood flow and increased pulmonary blood flow
a. Complete transposition of the great arteries
b. Double-outlet right ventricle of the Taussig-Bing type
c. Truncus arteriosus
d. Total anomalous pulmonary venous connection
e. Single ventricle without pulmonic stenosis
f. Common atrium
g. Tetralogy of Fallot
h. Tricuspid atresia with large VSD and no pulmonic stenosis
i. Hypoplastic left heart (aortic and mitral atresia)

II. Normal or Decreased pulmonary blood flow

a. Tricuspid atresia
b. Ebteins Anomaly with right to left atrial shunt
c. Pulmonary atresia with intact ventricular septum
d. Pulmonic stenosis or atresia with VSD (Tetralogy of Fallot)
e. Pulmonic stenosis with right to left atrial shunt TYPES OF ASD
f. Complete Transposition of the Great Arteries with
Pulmonic stenosis

ACYANOTIC WITH LEFT-TO-RIGHT SHUNT

Atrial Septal Defect

Most common CHD in Adults

Common; females > males
Magnitude of left-to-right shunt depends on:
1. Defect size
(the larger the defect size, the more blood going to the
other side)
2. Diastolic properties of both ventricles
A. Ostium Secundum anomalies
(if there is thickened ventricle, there is LESSER relaxation
Most common
of the ventricle.
Involves the fossa ovalis
In a heart with only a diastolic dysfunction, it will produce
Mid-septal in location
increased pressure within the cavity of the left ventricle,
complications will develop later. The filling pressure of
that left ventricle will increase the pressure within the
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 CLINICAL CHANGES IN THE HEART
1. Right atrial & ventricular enlargement
Due to increased blood volume to the right side of the
heart caused by left-to-right shunting of blood
2. Tricuspid regurgitation
TV stretched apart and may not close properly as theright
chambers enlarge blood leaks backward into right atrium
3. Pulmonary hypertension
Increase volume of blood flow to right side of heart
transmit increased pressure to lungs can lead to
B. Ostium primum anomalies
irreversible changes to pulmonary blood vessels
Lie immediately adjacent to atrioventricular valves, either of
4. Rhythm disturbances
which may be deformed & incompetent
Common in patients with Downs syndrome Enlargement of RA irritate sinus node atrial
2nd most common type arrhythmia
this type of ASD will have a mitral valve defect associated
with it called a mitral valve cleft, which is a slit-like or PHYSICAL EXAMINTATION
elongated hole in one of the leaflets (anterior) that form the 1. Prominent right ventricular impulse + palpable pulmonary
mitral valve artery pulsation
2. Normal or split S1; widely split S2 fixed in relation to respiration
3. Increased flow across pulmonic valve mid-systolic pulmonary
outflow murmur
4. Increased flow across TV mid-systolic diastolic rumbling
murmur, loudest at 4th ICS & along left sternal border
5. If with ostium primum defects apical thrill and holosystolic
murmur signify associated mitral or tricuspid incompetence or
a VSD
6. If increased pulmonary vascular resistance decreased L-to-R
shunt R-to-L shunt occurs
Decreased intensity of pulmonary & tricuspid murmurs
Accentuated pulmonic component of S2; accentuated
C. Sinus Venosus type systolic ejection murmur
Defect occurs high in the atrial septum near the entry of the Diastolic murmur of pulmonic regurgitation appears
superior vena cava and right atrium
May coexist with partial anomalous pulmonary venous DIAGNOSIS
connection of the right upper pulmonary vein 1. Electrocardiogram
Least common type a. Ostium Secundum
right axis deviation and an rSr pattern in right
precordial leads represent delayed activation of
venricular septum & enlargement of RV outflow tract
b. Sinus venosus type
first-degree heart block
c. Ostium primum
RV conduction defect accompanied by left axis
deviation

CLINICAL
Usually asymptomatic in early life
May have increased tendency for respiratory infections
Cardiorespiratory symptoms usually in older patients
Beyond 4th decade
Arrhythmias
Pulmonary arterial hypertension
Bi-directional and then right-to-left shunting of blood
Cardiac failure

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2. Chest X-ray
a. Right atrial and right ventricular enlargement
b. Dilatation of pulmonary artery and its branches
c. Increased pulmonary vascular markings
Xray chest PA view in atrial septal defect with severe pulmonary
hypertension. Prominent main pulmonary, right pulmonary artery
and left pulmonary artery
(behind the main pulmonary artery )
3. Echocardiogram
a. Pulmonary arterial and RV dilatation
b. Anterior systolic (paradoxical) or flat intrventricular septal
motion if with significant RV volume overload
c. Transesophageal echocardiography done if transthoracic
echo ambiguous usually in sinus venosus type
TREATMENT
1. Operative repair
a. Ideally in children 3 to 6 years old
b. Advised for all patients with uncomplicated secundum
ASD in whom there is significant L-to-r shunting
(pulmonary-to-systemic flow ratios approx. 2:1)
c. Not carried out in:
Patients with small defects & trivial L-to-R shunts
Patients with severe pulmonary vascular disease
without a significant L-to-R shunt
2. Medical management
a. Prompt treatment of respiratory tract infections
b. Anti-arrhytmic medications for AF or SVT
c. Management of hypertension, coronary disease, or heart
failure
ESSENTIALS OF DIAGNOSIS
Systolic murmur & fixed split S2 w/o cyanosis
Incomplete RBBB with RAD (secundum) & LAD (primum) on
ECG
Prominent PA & RV enlargement
RV dilatation, increased PA flow velocity & L-R atrial shunt by
Doppler
O2 step-up within RA; right-sided catheter can pass into the LA
across the defect
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Ventricular Septal Defect
Common as isolated defects and as one component of a
combination of anomalies
Opening usually single; located in membranous portion of the
septum
Functional disturbance dependent primarily on size of defect
and status of pulmonary vascular bed
Thats why on VSD you have a blood flow from RA,
RV, pulmonary artery, lungs and LA, LV and some of
this blood goes to RV.
VSD
CLINICAL
Range from spontaneous closure to CHF and death in early
infancy
Spontaneous closure in patients born with small defect and
occurs in early childhood
Possible development of pulmonary vascular obstruction
(Eisenmenger syndrome), RV outflow tract obstruction, aortic
regurgitation, and infective endocarditis
A lot of patient have spontaneous closure at around 5
yrs old. So later on VSD they are asymptomatic,
hinihintay nlng ng paediatrician na bka mgsara on its
own. 50-75% would close on its own.
Risk for developing pulmonary vascular obstruction highest in
patients with:
1. Large VSD
2. Pulmonary hypertension
Eisenmenger syndrome: exertional dyspnea, chest pain,
syncope, hemoptysis
R-to-L shunt -> clubbing of fingers, cyanosis, erythrocytosis
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 Clinical Symptoms:
Asymptomatic patient will later have pulmonary
hypertension because of inadequate oxygenation and
blood flow.
Then, they will complain of exertional dyspnea, chest
pain, syncope, hemoptysis. And clubbing of fingers,
cyanosis, erythrocytosis if the patient have right to left
shunt.
In all patients, degree to which pulmonary vascular resistance is
elevated before surgery is a critical factor for determining
prognosis
If pulmonary vascular resistance 1/3 or less of systemic value ->
progression of pulmonary vascular disease after surgery unusual
If moderate to severe pulmonary vascular resistance before
surgery no change or a progression of pulmonary vascular
disease common
DIAGNOSIS
1. Two-dimensional echocardiography with conventional or color
doppler examination
Define the number & location of defects & detect
associated anomalies
2. Hemodynamic & angiographic studies
Assess status of pulmonary vascular bed
TREATMENT
Surgery not recommended for patients with normal pulmonary
arterial pressures with small shunts
Since its anatomical defect, so surgery
Indication for surgery: moderate to large L-to-R shunt
ESSENTIALS OF DIAGNOSIS
History of murmur appearing shortly after birth
PE - Holosytolic murmur at LSB radiating right ward
PE LAE and LVE or biventricular enlargement
LAE, cant determine by PE
Present Left ventricular heaves and RV heaves.
Almost all of them have holosystolic thrill, on left lower
sternal border.
On auscultation there is systolic murmur on the left lower
sternal border, radiating right wards. Pag nilagay s kanan
walang murmur on tricuspid regurgitation.
Tricuspid regurgitation is a right sided murmur. So when
you ask the patient to inspiration it will increase intensity
of the murmur. Bec. Right sided murmur.
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VSD, systolic murmur, on left lower sternal border. Not
right sided murmur. Intensity of the murmur will not
increase in inspiration. (-) caravallos sign. murmur will
radiate to the right side.
Echocardiogram: will confirm VSD.
High velocity color flow Doppler jets across VSD
Increased pulmonary flow velocities
Patent Ductus Arteriosus
Ductus arteriosus
vessel leading from the bifurcation of the pulmonary
artery to the aorta just distal to left subclavian artery
Normally open on the fetal circulation
Closes immediately after birth
If not closed, PDA
Flow across ductus determined by:
Pressure and resistance relationships between the
systemic and pulmonary circulations
Theres a conduit between arterial and venous or
pulmonary circuit.
Pressure related between systemic and pulmonary
circulation, may tubo lang in between but theres still
circulation between aorta and pulmonary circulation.
Blood flow now on pulmonary artery to ductus to
descending aota or subclavian area.
If theres a reversal of blood flow,(ex, theres already a
pulmonary HTN). No unoxygenated blood flow on the
upper and theres unoxygenated down.
A. Normal anatomy of heart and blood flow of the interior of the
heart. B. Heart with a patent ductus arteriosus. The defect connects
the oarta with the pulmonary artery, allowing oxygen-rich blood
from the aorta to mix with oxygen poor blood in the pulmonary
artery
CLINICAL
Pulmonary pressures normal
Gradient and shunt from aorta to pulmonary artery persist
throughout cardiac cycle -> (+) thrill and continuous
machinery murmur with late systolic accentuation at upper
left sternal edge
continuous murmur, start at s1 and ends after s2 (even after s2
that still continuous murmur) blood flow from pulmonary
artery to aorta.
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 In normal pulmonary pressure, Blood flow from aorta to
pulmonary artery through ductus arteriosus.
even in diastole, the pressure in the aorta is higher than the
pulmonary pressure
so even in diastole, there is a blood flow coming from the aorta
to ductus to the pulmonary artery because there is contraction
and relax heart still there is continuous blood flow in blood
conduit. So there is continuous murmur.
diastole is the minimal pressure in conduit, arteries.
systole is diastole + cardiac output.

If with large L-to-R shunt through the ductus

Pulmonary vascular obstruction (Eisenmenger syndrome)
with pulmonary hypertension
R-to-L shunting develops
Cyanosis
If with severe pulmonary vascular disease -> reversal of flow
through ductus -> unoxygenated blood shunted to descending
aorta -> toes become cyanotic & clubbed (differential cyanosis)
During diastole the pulmonary artery pressure can now pass
the ductus to the aorta. Eh pababa so lower part of descending
part aorta supplies lower extremities, thats why you end up
with differential cyanosis. Where you appreciate cyanotic and
club toes on patient with PDA.
ASD and VSD, if with pulmonary arterial hypertension clubbed
and cyanotic upper and lower extremities.
Leading cause of death:
cardiac failure
and infective endocarditis
Severe pulmonary vascular obstruction may lead to aneurysmal
dilatation, calcifications and rupture of the ductus
to prevent cardiac failure in patient with PDA- SURGERY!
to prevent endocarditis 1). Wala ng primordial cause (tanggal
n yung ductus ee via surgery) 2). Prophylaxis
Chest Xray showing evidence of ductus arteriosus, as shown by the
enlargement of the aorta and pulmonary arteries in addition to left
ventricular dilatation and increased pulmonary vasculature
ESSENTIALS OF DIAGNOSIS
Continuous machinery like murmurs, loudest below the
clavicle.
LVH appreciated by Inspection, Palpation, Auscultation, ECG,
Chest Xrays and Echocardiogram.
Pulmonary plethora-> chest xray of patient with ductus
arteriosus
LVE & LAE and ductus arteriosus
Echocardiogram confirms diagnosis
Treatment:
1. Surgical ligation or division in the absence of severe
pulmonary vascular disease and predominant L-to-R
shunting
2. Transcatheter closure
3. Thoracoscopic surgical closure experimental

Acyanotic CHD without Shunt

Congenital Aortic Stenosis

Valvular Aortic Stenosis

3-4 time more common in males

Congenital bicuspid valves, which is not necessarily stenotic
one of the most common congenital malformations of the
heart -> may become stenosis with time -> may be site of
infective endocarditis
systolic regurgitation
Radiates to carotid

A. Echocardiogram in Apical four chamber view showing left

chamber clearly dilated with the interventricular septum shifted to
the right B. Suprasternal view showing turbulent blood flow from the
aorta (Ao) to the left pulmonary artery (LPA) through the ductus
arteriosus (PDA) RA- right atrium LA- left atrium; RV- right ventricle.
LV- left ventricle

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 Coarctation of the Aorta

Occurs in approx. 7% of patients with CHD

2x as common in males
Most frequent in patients with gonadal dysgenesis
Clinical manifestations depend on site and extent of
obstruction and presence of associated cardiac anomalies
(usually bicuspid aortic valve)
Secondary cause of Hypertension
XO (turners syndrome) most common with gonadal
dysgenesis, webbed neck.

The red arrows point to rib notching caused by the dilated intercostal
arteries. The yellow arrow points to the aortic knob, the blue arrow
to the actual coarctation and the green arrow to the post-stenotic
dilation of the descending aorta

3. MR angiography

CLINICAL
Most patients with isolated, discrete coarctation asymptomatic
Headache, epistaxis, cold extremities, claudication
CV manifestations:
Midsystolic murmur over anterior part of chest, back, and
spinous processes
Systolic and continuous murmurs over lateral thoracic wall
Hypertension in upper extremities
Decreased or delayed pulsation in femoral arteries
Enlarged and pulsatile collateral vessels in intercostals
spaces anteriorly (in the axillae) or posteriorly
(interscapular area)
Upper extremities and thorax more developed than the
lower extremities
Chicken feet
COMPLICATIONS
DIAGNOSIS 1. Cerebral aneurysm and hemorrhage
1. ECG LVH 2. Dissection and/or rupture of aorta
2. CXR 3. Premature coronary arteriosclerosis
a. Dilated left subclavian artery high on the left 4. LV failure
mediastinal border and dilated ascending aorta 5. Infective endocarditis
b. Indentation of aorta at site of coarctation with pre-
and post-stenotic dilatation (3 sign) TREATMENT
c. Notching of the ribs due to erosion by dilated
Surgical:
collateral vessels
a. Resection and end-to-end anastomosis
b. Subclavian flap angioplasty
c. Tubular graft, patch or bypass conduit if narrowed
segment is long
d. Percutaneous balloon dilatation commonly successful for
post-surgical re-coarctation

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 Fontan atriopulmonary or total cavopulmonary connection in
Essentials of diagnosis patients with normal or low pulmonary arterial resistance
Elevated systolic blood pressure in the upper extremities, pressure & good LV function
always in the right arm
normal systolic blood pressure on the lower extremities or Ebsteins Anomaly
hypotensive BP on lower extremities Anomalous attachment of tricuspid leaflets
There is a radial femoral pulse delay because of downward displacement of the TV into the RV
hypertension leads to tricuspid regurgitation
3 sign by Chest Xray RV often hypoplastic
LVH ECG, CXR Clinical manifestations variable
PE, History, ECG, Xray and echocardiogram Commonly associated defect is ASD and WPW.
WPW 25% associated with ebsteins but not all.
Pulmonary Stenosis with Intact Ventricular Septum Initial attention due to:
Valvular pulmonic stenosis the most common form of isolated 1. Progressive cyanosis from R-to-L shunting
RV obstruction 2. Symptoms of tricuspid regurgitation & RV dysfunction
Severity of obstructing lesion the most important determinant 3. Paroxysmal atrial tachyarrhtmias with or without
of clinical course atrioventricular bypass tracts (WPW syndrome)
Peak systolic transvalvular gradient of 50-80 mmHg moderate
stenosis COMMON ASSOCIATED CONDITIONS
>80 mmHg severe stenosis 1. ASD (50%)
<50 mmHg mild stenosis 2. Arryhtmias
systolic murmur 3. Wolff-parkinson-white syndrome(WPW)

CLINICAL 2-D echo: abnormal positional relation between tricuspid and

Cyanosis reflect R-to-L shunt through a patent foramen ovale mitral valves with apical displacement of septal tricuspid leaflet
or ASD Prosthetic replacement of tricuspid valve
In patients with severe pulmonary stenosis with CHF Used for diagnosis
may be accompanied by holosystolic decrescendo murmur
of tricuspid regurgitation TREATMENT
DIAGNOSIS Surgery
1. ECG assess degree of RV obstruction
a. Mild normal ECG
b. Moderate to severe right axis deviation, RVH
c. Severe ventricular strain pattern, high-amplitude P
waves in II and V1 (RA enlargement)
2. CXR RA & RV enlargement in severe stenosis with RV failure;
reduced pulmonary vascularity

Cyanotic CHD with Decreased Pulmonary blood flow

Tricuspid atresia
Characterized by:
1. Atresia of tricuspid valve
2. Interatrial communication
3. Hypoplasia of RV and pulmonary artery
CARPET heart of Ebsteins anomaly with marked cardiac
CLINICAL enlargement
severe cyanosis due to obligatory admixture of systemic and
pulmonary venous blood in LV Tetralogy of Fallot
most common cyanotic heart disease
DIAGNOSIS Components:
ECG: RA enlargement, LAD, LVH 1. Malaligned VSD
2. Obstruction to RV outflow (pulmonary stenosis)
3. Aortic override of the VSD
TREATMENT
4. RV hypertrophy
Atrial septostomy with anastomosis of a systemic artery or vein
PROV
to a pulmonary artery
P ulmonary valve stenosis
a. Increase pulmonary blood flow
R ight Ventricular hypertophy
b. Allow survival to the 2nd or 3rd decade
O verriding of aorta to VSD
V entricular Septal Defect

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Severity of RV outflow obstruction determines clinical
presentation; may be due to:
1. Pulmonary atresia
2. Pulmonary valve stenosis with supravalvular & peripheral
pulmonary arterial obstruction
3. Unilateral absence of pulmonary artery rare
In patient with tetralogy of fallot, the blood is coming from
RA, RV going to pulmonic valve.
The thing is pulmonic stenosis not allowing Blood flow from
right ventricle going to the right atrium??
So volume on RV will be decrease, as if there VSD it will go to
the left side now and contract. On contraction unoxygented
blood from left ventricle going to arterial circuit cyanosis
Therefore, Pulmonic stenosis is the cause of cyanosis
Overriding aorta
Saddles on both right and left ventricle
Unoxygenated blood goes to aorta thats why you have
cyanotic heart disease.
If no blood flow through the pulmonic valve because of
severe pulmonic stenosis walang ppuntang dugo para
maooxygenate, so ppunta s RV, VSD, LV contract heart s
LV so will have unoxygenated blood to the circulation
cyanosis.

Boot-shaped heart or Coeur en Sabot of TOF

there is uplifted apex and absence of pulmonary artery segment

Right-sided aortic arch & descending aorta in 25%

If with severe obstruction to RV outflow R-to-L shunting
occurs across the VSD severe cyanosis and erythrocytosis
with sequelae of systemic hypoxemia
In many infants and children, obstruction is mild but
progressive

Factors that may complicate treatment:

1. Infective endocarditis
2. Paradoxic embolism
3. Excessive erythrocytosis
4. Coagulation defects
Diagnosis: 5. Cerebral infarction or abscess
1. ECG RVH and less often, RA hypertrophy
2. CXR normal-sized boot-shaped heart (Coeur en sabot) with Corrective operation advisable for almost all patients
prominence of Rv and a concavity in the region of pulmonary Size of pulmonary arteries the most important determinant in
conus establishing candidacy for primary repair
3. 2-D echo demonstrate malaligntment of VSD and sub-
pulmonary stenosis TREATMENT: Surgery
4. Angiocardiography with RV injection architectural details of
RV outflow tract, pulmonary valve & annulus

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 Essentials of diagnosis
History of exercise intolerance & squatting
Central cyanosis, mild prominent RV impulse, pulmonic
stenosis on upper sternal border
Mild RVH
CXR-bootshaped heart
ECHO-confirm diagnosis

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OTHER FORMS OF CHD:
Congenitally corrected transposition

Two fundamental anatomic abnormalities:

1. Transposition of ascending aorta and pulmonary trunk
2. Inversion of the ventricles
De-saturated systemic venous blood: RA through MV LV
pulmonary trunk
Oxygenated pulmonary venous blood: LA through TV RV
aorta

Malpositions of the heart

Dextrocardia cardiac apex in the right side of the chest
Mesocardia cardiac apex in the midline of the chest
Isolated levocardia heart normally located in the left side of
chest but with abnormal position of the viscera

Dextrocardia with situs inversus. Cardiac apex is on the right side

and anatomical left lung in right hemithorax. Stomach is also on the
right side

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