You are on page 1of 12

adiuvante Dei gratia doctorum factionis 2014-2015

If there is Atrial Septal defect, if the pressure in right atrium is
NOBELANG INTROXD HIGHER than pressure in left atrium (usually, left atrium has
higher pressure as compared to right side) blood will flow from
Initially congenital heart disease will be tolerable but later it Right atrium to Left atrium, and that blood (unoxygenated) will
will end up with problems. go to the left ventricle then to the aorta then to systemic
Why are we studying these congenital heart diseases? circulation CYANOSIS
Because later, the less complex congenital heart And this condition is known as EISENMENGER SYNDROME
disease will have complications. *EISENMENGER SYNDROME
If you would notice the normal circulation: o the process in which a left to right shunt caused by
from SVC and IVC right atrium tricuspid valve a congenital heart defect in the fetal heart causes
right ventricle pulmonary valve pulmonary increased flow through the pulmonary vasculature,
causing pulmonary hypertension, which in turn causes
arteries lungs pulmonary veins left atrium
increased pressures in the right side of the heart and
mitral valve left ventricle aortic valve reversal of the shunt into a right-to-left shunt. (may
aorta reversal of shunt!)
If we would have a patient with Atrial Septal defect, the
the principal determinant of clinical manifestation for the
circulation is: feasibility of surgical repair is if the patient has an
right atrium tricuspid valve right ventricle uncomplicated congenital heart disease (Ex. ASD without PAH)
pulmonic valve pulmonary arteries lungs Imagine, the lungs of the patient is destroyed because of the
pulmonary veins left atrium some of the blood changes, but you can patch the hole in the intraatrial septum
goes back to right atrium due to a hole in the atrial using a pericadium patch using a device (yung ginawa kay kuya
septum (Initially) resulting in more blood volume in Kim, kaya nonexistent na yung ASD nya)
the right side of the heart, even in the pulmonary BUT if you do this surgery to a patient with PAH, USELESS LANG
circuit (there is VOLUME OVERLOAD) which may later ang closure of the intraatrial septum kasi nga fibrotic na yung
lead to complications caused by volume overload pulmonary vessels, mataas parin ang pressure sa pulmonary
such as pulmonary hypertension arteries, thus there will still be right sided heart failure UNLESS
you transplant new lungs to the patient.
COMPLICATIONS OF CHD: In the near future baka pwede na magpagawa ng lungs thru
1. PULMONARY ARTERIAL HYPERTENSION cloning (haha) then pwede na palitan yung baga. Magpagawa
2. EISENMENGER SYNDROME ka ng clone mo para hindi ka agad mamatay, papalit ka lang ng
3. ERYTHROCYTOSIS papalit ng defective organs mo. Unethical ba yun? pinapahaba
4. INFECTIVE ENDOCARDITIS mo lalo yung buhay? (harhar) etc. about cloning


For unknown reason, with that increase in pulmonary blood How do you diagnose Pulmonary Hypertension?
flow, you will end up having fibrotic changes within the o There is pressure within pulmonary artery, thats why
pulmonary parenchyma which will not allow the normal blood you call it PAH, and if that pressure is HIGH, what
flow coming from the pulmonary arteries to the pulmonary will happen to that pressure? It will go SOMEWHERE
arterial circuit, and if you have an abnormal blood flow, ELSE right ventricle right atrium right sided
pressure will increase within the pulmonary arteries heart failure manifestation (neck vein engorgement,
PULMONARY ARTERIAL HYPERTENSION (PAH) ascites, generalized edema) and erythrocytosis
hindi kasi makalusot yung dugo, dapat dadaan lang yung dugo Why Erythrocytosis?
sa pulmonary vessels, e kung di nakakalusot yung dugo, anong o In ASD with pulmonary hypertension (Eisenmenger
mangyayari sa volume and pressure within the pulmonary syndrome), wherein the right atrial pressure is higher
artey?within the right ventricle? Within the right atrium? than left atrial pressure (so blood flows from right to
The first thing that will happen is we end up having PAH left atrium), UNOXYGENATED blood is being pumped
So when there is PAH, blood pressure is higher as compared to out of arterial circuit (dahil may reversal of shunt),
right ventricular pressure resulting to back flow of blood from there will be hypoxemic blood in the left ventricle and
pulmonary arteries to right ventricle then to right atrium (which in the arterial circuit which be sensed as HYPOXIA by
has lesser pressure) the body. When the body senses hypoxia, it will
stimulate the kidneys to produce erythropoeitin
inc. RBC production ERYTHROCYTOSIS.

If youre tired.SLEEP
Let your FAITH be bigger than your fear
Page 1 of 12 EUNICE and
o Then pag sobra yung RBCs, patient is more prone to o For endocarditis to develop, two independent events
develop thrombosis (thats why you do phlebotomy are normally required:
in patients with very high Hgb and Hct) an area of endothelium must be damaged,
On the other hand, in pateints with Tetralogy of Fallot, syempre and a bacteremia caused by adherent
hypoxemic na yung patient, you give aspirin (antithrombotic), organisms must occur. (AHA)
but they have paradoxical bleeding (for unknown reasons), ASD- MOST COMMON CHD in ADULTS
they have a tendency to bleed, but we still give aspirin for the VSD- MOST COMMON CHD for ALL AGES
viscosity of the patients blood kaya mejo mahirap din yung pag
balance ng pag bigay ng aspirin in a patient with hypoxia EPIDEMIOLOGY
secondary to congenital heart disease.
1% of all live births
4. INFECTIVE ENDOCARDITIS 4% of offspring of women with congenital heart disease
Patients with CHD are prone to develop INFECTIVE Generally the result of aberrant embryonic development of a
ENDOCARDITIS. normal structure or failure of such a structure to progress
INFECTIVE ENDOCARDITIS beyond an early stage of embryonic or fetal development
o Infection of the endocardium Complex multifactorial genetic and environmental causes
o Microbacterium is inside the endocardium, can you Chromosomal aberrations and mutations of single genes
imagine, for EVERY cardiac contraction there would account for 10% of all cardiac malformations
be bacteria released in the circulation bacteremia
o if it goes to the brain brain abscess CLASSIFICATION OF CHD
o if it goes to the eyes blindness
o if it goes to the heart coronary artery infection A. ACYANOTIC with Left-to-Right shunt
So patients with Congenital Heart Disease should have
Infective Endocarditis Prophylaxis (IE prophylaxis) especially I. Atrial level shunt
those undergoing dental procedures, GUT and GIT diagnostic a. Atrial septal defect (ASD)
procedures. Give antibiotic therapy to ALL patients with b. Atrial septal defect with mitral stenosis (Lutembachers
valvular heart disease prior to these procedures EXCEPT FOR: syndrome)
o ASD, ostium Secundum type c. Partial anomalous pulmonary venous connection
o Pure mitral valve prolapse
For the rest, or If youre not sure, just give your IE prophylaxis. II. Ventricular level shunt
For dental procedures: give oral Amoxicillin (3 grams prior to a. Ventricular septal defect (VSD)
procedure then 1.5 grams after the procedure b. VSD with aortic regurgitation
So pag may murmur ang patient, magbigay nalang ng IE c. VSD with left ventricular to right atrial shunt
Other measures: III. Aortic root to right heart shunt
o clean teeth a. Ruptured sinus of Valsalva aneurysm
o dental abscesses must be removed b. Coronary arteriovenous fistula
Does brushing of teeth produce bacteremia? c. Anomalous origin of the left coronary artery from the
o Common oral hygiene, such as brushing teeth or pulmonary trunk
flossing, can cause transient but harmless bacteremia.
Some patients with prosthetic heart valves however IV. Aortopulmonary level shunt
need antibiotic prophylaxis for dental surgery because a. Aortopulmonary window
bacteremia might lead to endocarditis (Wikipedia) so b. Patent ductus arteriosus
Infective Endocarditis V. Multiple level shunts
o While anyone can contract endocarditis, the infection a. Complete common atrioventricular canal
usually develops in individuals with underlying b. VSD with ASD
structural cardiac defects. It can occur whenever c. VSD with PDA
these persons develop bacteremia with the organisms
likely to cause endocarditis. Bacteremia may occur B. ACYANOTIC without a Shunt
spontaneously (i.e., caused by organisms introduced
through food chewing or tooth brushing), or it may Here, the problem is MALDEVELOPMENT of certain structures.
develop as a complication of a focal infection, such as For example:
a periodontal or periapical infection, a urinary tract a fibrotic lesion in the pulmonary veins at birth
infection, pneumonia or cellulitis. Pulmonary vein stenosis (also a CHD)
o Selected surgical and dental procedures and a deformed mitral valve after birth mitral stenosis
instrumentations, especially those involving mucosal problem secondary to CHD
surfaces or contaminated tissue, can cause a transient
bacteremia that rarely persists for more than a few

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 2 of 11 EUNICE and
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


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
If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 3 of 11 EUNICE and
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
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

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

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 4 of 11 EUNICE and
2. Chest X-ray Ventricular Septal Defect
a. Right atrial and right ventricular enlargement Common as isolated defects and as one component of a
b. Dilatation of pulmonary artery and its branches combination of anomalies
c. Increased pulmonary vascular markings Opening usually single; located in membranous portion of the
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.

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 VSD
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

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 Range from spontaneous closure to CHF and death in early
(pulmonary-to-systemic flow ratios approx. 2:1) infancy
c. Not carried out in: Spontaneous closure in patients born with small defect and
Patients with small defects & trivial L-to-R shunts occurs in early childhood
Patients with severe pulmonary vascular disease Possible development of pulmonary vascular obstruction
without a significant L-to-R shunt (Eisenmenger syndrome), RV outflow tract obstruction, aortic
2. Medical management regurgitation, and infective endocarditis
a. Prompt treatment of respiratory tract infections A lot of patient have spontaneous closure at around 5
b. Anti-arrhytmic medications for AF or SVT yrs old. So later on VSD they are asymptomatic,
c. Management of hypertension, coronary disease, or heart hinihintay nlng ng paediatrician na bka mgsara on its
failure own. 50-75% would close on its own.

Risk for developing pulmonary vascular obstruction highest in

patients with:
Systolic murmur & fixed split S2 w/o cyanosis 1. Large VSD
Incomplete RBBB with RAD (secundum) & LAD (primum) on 2. Pulmonary hypertension
Prominent PA & RV enlargement Eisenmenger syndrome: exertional dyspnea, chest pain,
RV dilatation, increased PA flow velocity & L-R atrial shunt by syncope, hemoptysis
Doppler R-to-L shunt -> clubbing of fingers, cyanosis, erythrocytosis
O2 step-up within RA; right-sided catheter can pass into the LA
across the defect

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 5 of 11 EUNICE and
Clinical Symptoms: VSD, systolic murmur, on left lower sternal border. Not
Asymptomatic patient will later have pulmonary right sided murmur. Intensity of the murmur will not
hypertension because of inadequate oxygenation and increase in inspiration. (-) caravallos sign. murmur will
blood flow. radiate to the right side.
Then, they will complain of exertional dyspnea, chest Echocardiogram: will confirm VSD.
pain, syncope, hemoptysis. And clubbing of fingers, High velocity color flow Doppler jets across VSD
cyanosis, erythrocytosis if the patient have right to left Increased pulmonary flow velocities
In all patients, degree to which pulmonary vascular resistance is Patent Ductus Arteriosus
elevated before surgery is a critical factor for determining Ductus arteriosus
prognosis vessel leading from the bifurcation of the pulmonary
If pulmonary vascular resistance 1/3 or less of systemic value -> artery to the aorta just distal to left subclavian artery
progression of pulmonary vascular disease after surgery unusual Normally open on the fetal circulation
If moderate to severe pulmonary vascular resistance before Closes immediately after birth
surgery no change or a progression of pulmonary vascular
If not closed, PDA
disease common
Flow across ductus determined by:
DIAGNOSIS Pressure and resistance relationships between the
1. Two-dimensional echocardiography with conventional or color systemic and pulmonary circulations
doppler examination Theres a conduit between arterial and venous or
Define the number & location of defects & detect pulmonary circuit.
associated anomalies Pressure related between systemic and pulmonary
2. Hemodynamic & angiographic studies circulation, may tubo lang in between but theres still
Assess status of pulmonary vascular bed 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.

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
A. Normal anatomy of heart and blood flow of the interior of the
ESSENTIALS OF DIAGNOSIS heart. B. Heart with a patent ductus arteriosus. The defect connects
History of murmur appearing shortly after birth the oarta with the pulmonary artery, allowing oxygen-rich blood
PE - Holosytolic murmur at LSB radiating right ward from the aorta to mix with oxygen poor blood in the pulmonary
PE LAE and LVE or biventricular enlargement artery
LAE, cant determine by PE
Present Left ventricular heaves and RV heaves. CLINICAL
Almost all of them have holosystolic thrill, on left lower Pulmonary pressures normal
sternal border. Gradient and shunt from aorta to pulmonary artery persist
On auscultation there is systolic murmur on the left lower throughout cardiac cycle -> (+) thrill and continuous
sternal border, radiating right wards. Pag nilagay s kanan machinery murmur with late systolic accentuation at upper
walang murmur on tricuspid regurgitation. left sternal edge
Tricuspid regurgitation is a right sided murmur. So when continuous murmur, start at s1 and ends after s2 (even after s2
you ask the patient to inspiration it will increase intensity that still continuous murmur) blood flow from pulmonary
of the murmur. Bec. Right sided murmur. artery to aorta.

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 6 of 11 EUNICE and
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 Chest Xray showing evidence of ductus arteriosus, as shown by the
If with severe pulmonary vascular disease -> reversal of flow enlargement of the aorta and pulmonary arteries in addition to left
through ductus -> unoxygenated blood shunted to descending ventricular dilatation and increased pulmonary vasculature
aorta -> toes become cyanotic & clubbed (differential cyanosis)
During diastole the pulmonary artery pressure can now pass ESSENTIALS OF DIAGNOSIS
the ductus to the aorta. Eh pababa so lower part of descending Continuous machinery like murmurs, loudest below the
part aorta supplies lower extremities, thats why you end up clavicle.
with differential cyanosis. Where you appreciate cyanotic and LVH appreciated by Inspection, Palpation, Auscultation, ECG,
club toes on patient with PDA. Chest Xrays and Echocardiogram.
ASD and VSD, if with pulmonary arterial hypertension clubbed Pulmonary plethora-> chest xray of patient with ductus
and cyanotic upper and lower extremities. arteriosus
Leading cause of death: LVE & LAE and ductus arteriosus
cardiac failure Echocardiogram confirms diagnosis
and infective endocarditis
Severe pulmonary vascular obstruction may lead to aneurysmal Treatment:
dilatation, calcifications and rupture of the ductus 1. Surgical ligation or division in the absence of severe
to prevent cardiac failure in patient with PDA- SURGERY! pulmonary vascular disease and predominant L-to-R
to prevent endocarditis 1). Wala ng primordial cause (tanggal shunting
n yung ductus ee via surgery) 2). Prophylaxis 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

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 7 of 11 EUNICE and
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

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
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
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

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 8 of 11 EUNICE and
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
>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
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
4. RV hypertrophy
Atrial septostomy with anastomosis of a systemic artery or vein
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

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 9 of 11 EUNICE and
Severity of RV outflow obstruction determines clinical Overriding aorta
presentation; may be due to: Saddles on both right and left ventricle
1. Pulmonary atresia Unoxygenated blood goes to aorta thats why you have
2. Pulmonary valve stenosis with supravalvular & peripheral cyanotic heart disease.
pulmonary arterial obstruction If no blood flow through the pulmonic valve because of
3. Unilateral absence of pulmonary artery rare severe pulmonic stenosis walang ppuntang dugo para
In patient with tetralogy of fallot, the blood is coming from maooxygenate, so ppunta s RV, VSD, LV contract heart s
RA, RV going to pulmonic valve. LV so will have unoxygenated blood to the circulation
The thing is pulmonic stenosis not allowing Blood flow from cyanosis.
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

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

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

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 10 of 11 EUNICE and
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

God bless us sa finals! One last push, lets do

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

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

If youre tired.SLEEP
Let your FAITH be bigger than your fear Page 11 of 11 EUNICE and