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CARDIO: DR.
BARTOLOME
CONGENITAL HEART DISEASES
CONGENITAL HEART DISEASE 2. EISENMENGER SYNDROME
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
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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
prophylaxis
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
YES.
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
minutes.
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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)
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 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
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.
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
TREATMENT
1. Operative repair
a. Ideally in children 3 to 6 years old
CLINICAL
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.
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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
shunt.
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.
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
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.
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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.
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Coarctation of the Aorta
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)
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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
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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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