MEDICINE (CARDIOLOGY)

Topic: Congenital Heart Disease

Lecturer: Dr. Jerelyn Adviento
Reference: Harrisonss Principles of Internal Medicine
CONGENITAL HEART DISEASE IN
THE ADULT
Cardiac Development
CHD is generally the result of aberrant embryonic development of a normal structure or failure of such structure to
progress beyond an early stage of embryonic or fetal development.

Cardiogenesis finely tuned process with transcriptional control of a complex group of regulatory proteins that
activate or inhibit their gene targets in a location- and time-dependent manner

Specific Cardiac Defects

Simple defects= single lesions with a shunt or a valvular malformation

Intermediate defects= have two or more simple defects

Complex defects= intermediate defect plus more complex cardiac and vascular anatomy, often with cyanosis, and
frequently with transposition complexes

Atrial Septal Defect

Common cardiac anomaly
Occurs frequently in females
Sinus venosus ASD
o occurs high in the atrial septum near the entry
of the superior vena cava into the right atrium

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 o associated frequently with anomalous pulmonary venous connection from the right lung to the superior
vena cava or right atrium
Ostium primum ASD
o lie adjacent to the atrioventricular valves, either of which may be deformed and regurgitant
o common in Downs syndrome, often as part of complex AV septal defects with a common AV valve and
a posterior defect of the basal portion of the interventricular septum
Ostium segundum ASD
o Most commonly involves the fossa ovalis and is midseptal in location
ASD denotes a true deficiency of the atrial septum and implies functional and anatomic patency
Factors affecting left-to-right shunt
o ASD size
o Ventricular diastolic properties
o Relative impedance
Left-to-right shunt causes diastolic overloading of the RV and increased pulmonary blood flow
Patients are asymptomatic in early life
Cardiorespiratory symptoms occur in many older patients
Beyond 4th decade atrial arrhythmias, pulmonary arterial hypertension, right heart failure
In older patients, left-to-right shunting across the ASD increases as progressive systemic hypertension and/or
coronary artery disease (CAD) result in reduced compliance of the LV
Physical Examination
prominent RV impulse
palpable pulmonary artery pulsation
First heart sound
o normal or split, with accentuation of the tricuspid valve closure sound
o midsystolic pulmonary outflow murmur Increased flow across the pulmonic valve
Second heart sound
o widely split and is fixed in relation to respiration
o mid-diastolic rumbling murmur, loudest at the fourth intercostal space and along the left sternal border
reflects increased flow across the tricuspid valve
In ostium primum ASD
o an apical holosystolic murmur indicates associated mitral or tricuspid regurgitation or a ventricular
septal defect (VSD)
Increased pulmonary vascular resistance causes diminution of the left-to-right shunt
o pulmonary outflow and tricuspid inflow murmurs decrease in intensity
o pulmonic component of the second heart sound and a systolic ejection sound are accentuated
o diastolic murmur of pulmonic regurgitation appears
Cyanosis and clubbing accompany the development
of a right-to-left shunt
adults with an ASD and atrial fibrillation
o findings may be confused with mitral stenosis with pulmonary hypertension
o tricuspid diastolic flow murmur and widely
split second heart sound may be mistakenly thought to represent the diastolic murmur of mitral
stenosis and the mitral opening snap, respectively
Electrocardiogram
Ostium segundum ASD
o Shows right-axis deviation
o rSr pattern in the right precordial leads representing enlargement of the RV outflow tract
Sinus venous ASD
o Ectopic atrial pacemaker or first-degree heart block
Ostium primum ASD
o Left superior axis deviation
o Counterclockwise rotation of the frontal plane QRS loop
Varying degrees of RV and right atrial (RA)
enlargement or hypertrophy may occur with each
type of defect, depending on the presence and
degree of pulmonary hypertension
CXR: enlarged RA and RV and pulmonary artery and its branches
Echocardiogram

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 reveals pulmonary arterial and RV and RA dilatation with abnormal (paradoxical) ventricular septal motion in
the presence of a significant right heart volume overload
Visualization of the ASD
o Two-dimensional imaging
o Color-flow imaging
o Echocontrast
Echocardiography and Doppler examination
supplanted cardiac catheterization
Transesophageal echocardiography
o indicated if the transthoracic echocardiogram is ambiguous - the case with sinus venosus defects, or
for guiding catheter device closure
Cardiac catheterization
o Criteria:
inconsistencies exist in the clinical data,
significant pulmonary hypertension or associated malformations are suspected,
CAD is a possibility
when attempting transcatheter closure of the ASD

Treatme ATRIAL SEPTAL DEFECT

nt
Operative Repair
usually with a patch of pericardium or of prosthetic
material or percutaneous transcatheter device closure
Pulmonary vasodilators
reduction in pulmonary artery pressure and resistance may allow closure of ASD in patients with
pulmonary vascular disease
Patients with sius venosus or ostium secundum
o Medical management should include prompt treatment of respiratory tract infections; antiarrhythmic
medications for atrial fibrillation or supraventricular tachycardia; and the usual measures for
hypertension, coronary disease, or heart failure

Ventricular Septal Defect

One of the most common of all cardiac birth defects
Usually single or situated in the membranous or midmuscular portion of the septum
Functional disturbance depends on its size and on the status of the pulmonary vascular bed
Small-or moderate-size VSDs adulthood
Large VSD early in life
VSD Spectrum
o Spontaneous closure
More common in patients born with a small VSD
occurs in early childhood in most
o Congestive cardiac failure
o Death
o Development of pulmonary vascular obstruction

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 Principal determinant of the clinical manifestations and course of a given VSD and feasibility of
surgical repair
o RV outflow tract obstruction
Results to increased pulmonary arterial pressure
Result of obstructive, obliterative structural changes within the pulmonary vascular bed
develops in ~510% of patients who present in infancy with a moderate to large left-to-right shunt
o Aortic regurgitation
~5% of patients, results from insufficient cusp tissue or prolapse of the cusp through the
interventricular defect
o Infective endocarditis
Eisenmengers syndrome
o patients with a large communication between the two circulations at the aortopulmonary, ventricular,
or atrial levels and bidirectional or predominantly right-to-left shunts because of high resistance and
obstructive pulmonary hypertension
o exertional dyspnea, chest pain, syncope and hemoptysis
o pregnancy is contraindicated
large VSDs and pulmonary hypertension greatest risk for developing pulmonary vascular disease
right-to-left shunt leads to cyanosis, clubbing and erythrocytosis
Echocardiography with spectral and color Doppler examination
defines the number and location of defects in the ventricular septum and associated anomalies and the
hemodynamic physiology of the defects
Hemodynamic and angiographic study
may be occasionally required to assess the status of the pulmonary vascular bed and clarify details of the
altered anatomy
computed tomography (CT) or magnetic resonance imaging (MRI)
useful in delineating complex anatomy and assessing extracardiac structures
Treatme VENTRICULAR SEPTAL DEFECT
nt
Closure
Not recommended for patient with normal pulmonary arterial pressures with small shunts (pulmonary-to-
systemic flow ratios of <1.5:1)
Operative correction or transcatheter closure
indicated when there is a moderate to large left-to-right shunt with a pulmonary-to-systemic flow ratio
>1.5:1
in the absence of prohibitively high levels of pulmonary vascular resistance (pulmonary arterial
resistance is less than two-thirds of systemic arterial resistance)
Patent Ductus Arteriosus (PDA)
Ductus Arteriosus: a vessel leading from the bifurcation of the pulmonary artery to the aorta just distal to
the left subclavian artery
Vascular channel is open in the fetus but closes immediately after birth
In most adults, pulmonary pressures are normal, and a gradient and shunt from aorta to pulmonary artery
persist throughout the cardiac cycle, resulting in a characteristic thrill and a continuous machinery
murmur with late systolic accentuation at the upper left sternal edge
Adult with large left-to-right shunt through ductus arteriosus
o pulmonary vascular obstruction (Eisenmengers syndrome) with pulmonary hypertension,
right-to-left shunting, and cyanosis have usually developed
Severe pulmonary vascular disease results in reversal of flow through the ductus;
unoxygenated blood is shunted to the descending aorta;
the toesbut not the fingersbecome cyanotic and clubbed, a finding termed differential cyanosis
Cardiac Failure and Infective Endocarditis leading causes of death in adults with PDA
Severe Pulmonary Vascular Obstruction: may
cause aneurysmal dilatation, calcification, and rupture of the ductus

Aortic Root-to-Right Shunts

Three most common causes:
(1) congenital aneurysm of an aortic sinus of Valsalva with fistula

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 (2) coronary arteriovenous fistula
(3) anomalous origin of the left coronary artery from the pulmonary trunk
Aneurysm of an aortic sinus of Valsalva
separation or lack of fusion between the media of the aorta and the annulus of the aortic valve
Rupture occurs in the third or fourth decade of life
Aorticocardiac fistula is between the right coronary cusp and the RV; but occasionally, when the noncoronary
cusp is involved, the fistula drains into the RA
Abrupt rupture causes chest pain, bounding pulses, a continuous murmur accentuated in diastole,
and volume overload of the heart
Diagnosis:
2-D and Doppler echocardiographic studies
Cardiac catheterization- quantifies left-to-right shunt
Thoracic aortography- visualizes fistula
Medical management directed at cardiac failure, arrhythmias or endocarditis
At operation, the aneurysm is closed and amputated, and the aortic wall is reunited with the heart, either by
direct suture or with a patch or prosthesis
Coronary Arteriovenous Fistula
Unusual anomaly
consists of a communication between a coronary artery and another cardiac chamber, usually the coronary
sinus, RA, or RV
shunt is usually of small magnitude, and myocardial blood flow is not usually compromised
if shunt is large, there may be a coronary steal syndrome with myocardial ischemia and possible angina or
ventricular arrhythmias
PE: loud, superficial, continuous murmur at the lower or midsternal border usually prompts a further
evaluation of asymptomatic patient
Diagnosis:
Doppler echocardiography: demonstrate site of drainage
2D echocardiography: detected if site of origin is proximal
Angiography (classic catheterization, CT, or MRI): permits identification of the size and anatomic features
of the fistulous tract, may be closed by suture or transcatheter obliteration
Anomalous origin of the LCA from the pulmonary artery
oxygenated blood from the aortic root flows via a dilated RCA and collaterals to the LCA and retrograde to the
lower pressure pulmonary artery circulation via the anomalous LMCA (which emerges from the pulmonary
artery)
Diagnosis: ECG findings of an anterolateral myocardial infarction and left ventricular hypertrophy (LVH)
Management: coronary artery reimplantation, coronary artery bypass with an internal mammary artery graft,
or saphenous vein-coronary artery graft
Congenital Aortic Stenosis
Malformation that cause obstruction to LV outflow
(a) Congenital valvular aortic stenosis
(b) Discrete subaortic stenosis
(c) Supravalvular aortic stenosis
Bicuspid aortic valves are more common in males than in females
Congenital bicuspid aortic valve initially function normal, most common congenital malformations of the
heart and may go undetected in early life
May develop stenosis or regurgitation with time or be the site of infective endocarditis= difficult to distinguish
from acquired rheumatic or degenerative calcific aortic valve disease
o congenitally deformed, rigid aortic valve commonly lead to thickening of the cusps and, in later life, to
calcification
Obstruction = concentric hypertrophy of the LV wall
Diagnosis: Echocardiography reveals morphology of the aortic valve and aortic root and quantitates
severity of stenosis or regurgitation
Treatme VALVULAR AORTIC STENOSIS
nt
Patient with diminished cardiac reserve
Administration of Digoxin, Diuretics and Sodium restriction while awaiting operation
Dilated aortic root
Beta blockers, ARBs, or ACE inhibitors
Aortic valve replacement
Indicated in adults with critical obstruction
i.e., aortic valve area <0.45 cm2/m2, with symptoms secondary to LV dysfunction or myocardial ischemia,
or with hemodynamic evidence of LV dysfunction
Aortic balloon valvuloplasty

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 asymptomatic children or adolescents or young adults with critical aortic stenosis without valvular
calcification or these features
If surgery is contraindicated in older patients because of a complicating medical problem such as
malignancy or renal or hepatic failure, balloon valvuloplasty may provide short-term improvement
Serve as bridge to aortic valve replacement in patients with severe HF
Transcatheter aortic valve replacement
Potential alternative to surgery
Subaortic Stenosis
discrete form is consists of a membranous diaphragm or fibromuscular ring encircling the LV outflow tract just
beneath the base of the aortic valve.
The jet impact from the subaortic stenotic jet on the underside of the aortic valve often begets progressive
aortic valve fibrosis and valvular regurgitation.
Diagnosis:
Echocardiography demonstrates the anatomy of the subaortic obstruction
Doppler studies show turbulence proximal to the aortic valve and can quantitate the pressure gradient and
severity of aortic regurgitation
Treatment: complete excision of the membrane or fibromuscular ring
Supravalvular Aortic Stenosis
localized or diffuse narrowing of the ascending aorta originating just above the level of the coronary arteries at
the superior margin of the sinuses of Valsalva
the coronary arteries are subjected to elevated systolic pressures from the LV, are often dilated and tortuous,
and are susceptible to premature atherosclerosis.
The coronary ostia may also become obstructed by the aortic valve leaflets
a genetic defect is located in chromosome region as elastin on chromosome 7
most commonly associated cardiac defect in Williams-Beuren syndrome
elfin facies
low nasal bridge
cheerful demeanor
mental retardation with retained language skills and love of music
supravalvular aortic stenosis, and
transient hypercalcemia
Coarctation of the Aorta
Narrowing or constriction of the lumen of the aorta
may occur anywhere along its length but is most common distal to the origin of the left subclavian artery near
the insertion of the ligamentum arteriosum
occurs in ~7% of patients with CHD
more common in males than females,
particularly frequent in patients with gonadal dysgenesis (e.g., Turners syndrome).
Clinical manifestations depend on: (1) site and (2) extent of obstruction and (3) the presence of associated
cardiac anomalies, most commonly a bicuspid aortic valve.
Circle of Willis aneurysms may occur in up to 10%
Headache, epistaxis, chest pressure, and claudication with exercise may occur
Physical Examination
attention is usually directed to the cardiovascular system when a heart murmur or hypertension in the upper
extremities and absence, marked diminution, or delayed pulsations in the femoral arteries are detected on
physical examination
Enlarged and pulsatile collateral vessels may be palpated in the intercostal spaces anteriorly, in the axillae, or
posteriorly in the interscapular area
A midsystolic murmur over the left interscapular space may become continuous if the lumen is narrowed
sufficiently to result in a high-velocity jet across the lesion throughout the cardiac cycle
Additional systolic and continuous murmurs over the lateral thoracic wall may reflect increased flow through
dilated and tortuous collateral vessels
ECG LV hypertrophy
Chest X-ray dilated left subclavian artery high on the left mediastinal border and a dilated ascending aorta
Indentation of the aorta at the site of coarctation and pre- and poststenotic dilatation (the 3 sign) along the
left paramediastinal shadow are essentially pathognomonic
Notching of the third to ninth ribs- radiographic sign-due to inferior rib erosion by dilated collateral vessels
2D-echocardiography from suprasternal windows identifies the site of coarctation
Doppler quantitates the pressure gradient
Transesophageal echocardiography and MRI or CT allow visualization of the length and severity of the
obstruction and associated collateral arteries.
Cardiac catheterization: indicated primarily to evaluate the coronary arteries or to perform catheter-based
intervention (angioplasty and stent of the coarctation) in adults
Chief hazards of proximal aortic severe hypertension
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 (a) cerebral aneurysms and hemorrhage
(b) aortic dissection and rupture
(c) premature coronary arteriosclerosis
(d) aortic valve failure
(e) LV failure;
infective endarteritis may occur on the coarctation site or
endocarditis may settle on an associated bicuspid aortic valve (~50% of patients)
Treatme COARCTATION OF THE AORTA
nt
Surgical or involves percutaneous catheter balloon
dilatation with stent placement
high-quality MRI or CT procedure in follow-up
Pulmonary Stenosis with Intact Ventricular Septum
Obstruction to RV outflow may be localized to the supravalvular, valvular, or subvalvular levels or occur at
a combination of these sites
Multiple sites of narrowing of the peripheral pulmonary arteries are a feature of rubella embryopathy and may
occur with both the familial and sporadic forms of supravalvular aortic stenosis
Valvular pulmonic stenosis (PS): most common form of isolated RV obstruction
Severity of the obstructing lesion most important determinant of the clinical course
In the presence of a normal cardiac output
o <30 mmHg indicates mild PS
generally asymptomatic and demonstrate little or no progression in the severity of obstruction
with age
o >50 mmHg indicates severe PS
Symptoms vary with the degree of obstruction
o Fatigue, dyspnea, RV failure, and syncope may limit the activity of older patients moderate or severe
obstruction may prevent an augmentation of cardiac output with exercise
o patients with severe obstructionthe systolic pressure in the RV may exceed that in the LV, because
the ventricular septum is intact
o moderate or severe stenosis
RV ejection is prolonged
the sound of pulmonary valve closure is delayed and soft
o RV hypertrophy reduces the compliance of that chamber, and a forceful RA contraction is necessary to
augment RV filling
o A fourth heart sound
o prominent a waves in the jugular venous pulse
o occasionally, presystolic pulsations of the liver reflect vigorous atrial contraction
Clinical diagnosis
o left parasternal lift and harsh systolic crescendo-decrescendo murmur
o thrill at the upper left sternal border, typically preceded by a systolic ejection sound if the obstruction
is due to a mobile nondysplastic pulmonary valve
o holosystolic murmur of tricuspid regurgitation may accompany severe PS, especially in the presence of
congestive heart failure
o Cyanosis right-to-left shunting through a patent foramen ovale or ASD
o Supravalvular or peripheral pulmonary arterial stenosismurmur is systolic or continuous and is best
heard over the area of narrowing, with radiation to the peripheral lung fields
In mild cases,
o the ECG is normal, whereas moderate and severe stenoses are associated with RV hypertrophy
o chest x-ray with mild or moderate PS shows a heart of normal size with normal lung vascularity.
Pulmonary valvular stenosis,
o dilatation of the main and left pulmonary arteries occurs in part due to the direction of the PS jet and in
part due to intrinsic tissue weakness
severe obstructionRV hypertrophy
The pulmonary vascularity may be reduced with
o severe stenosis,
o RV failure, and/or
o right-to-left shunt at the atrial level.
2D/3D echocardiographyvisualizes pulmonary valve morphology
Doppler echocardiographyquantify the outflow tract pressure gradient (Fig. 282-4)

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Treatme PULMONARY STENOSIS
nt
Cardiac catheter technique of balloon
valvuloplasty
effective, and the surgery is rarely necessary
Transcatheter Angioplasty or Stenting
Treats effectively the Multiple stenoses of the
peripheral pulmonary arteries
TETRALOGY OF FALLOT
Four components:
1. Malaligned VSD
2. Obstruction to RV outflow
o Severity Determines the clinical presentation
o Severity of hypoplasia of the tract varies from mild to complete (pulmonary atresia)
3. Aortic override of the VSD
4. RV hypertrophy
o due to the RVs response to aortic pressure via the large VSD
Pulmonary valve stenosis and supravalvular and peripheral pulmonary arterial obstruction may coexist
Unilateral absence of a pulmonary artery -usually the left(RARE)
Right-sided aortic arch and descending thoracic aorta occur in ~25%
Severe RV outflow obstruction
o pulmonary blood flow is reduced
o large volume of desaturated systemic venous blood shunts right-to-left across the VSD
o Severe cyanosis and erythrocytosis
o symptoms of systemic hypoxemia are prominent
In many infants and children, the obstruction is mild but progressive
Diagnosis:
ECG showsRV hypertrophy
Chest x-ray
o shows a normal-sized, boot-shaped heart (coeur en sabot) with a prominent RV and a concavity in the
region of the pulmonary conus
o Pulmonary vascular markings are typically diminished, and the aortic arch and knob may be on the right
side
Echocardiography
o demonstrates the malaligned VSD with the overriding aorta
o the site and severity of PS, which may be subpulmonic (fixed or dynamic), at the pulmonary valve or in the
main or branch pulmonary arteries
Classic contrast angiography
o may provide details regarding the RV outflow tract, pulmonary valve and annulus, and caliber of the main
branches of the pulmonary artery, as well as about possible associated aortopulmonary collaterals.
Coronary arteriography
o identifies the anatomy and course of the coronary arteries, which may be anomalous
Cardiac MRI and CT complement echocardiography
o provide much of the information gathered by angiography as well as additional functional information.
o MRI is considered the clinical gold standard for quantification of RV volume and function as well as
quantification of the pulmonary regurgitation severity

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Treatme Tetralogy of Fallot
nt
Reoperation in adults is most commonly for
severe pulmonary regurgitation or pulmonary
stenosis
Ventricular and atrial arrhythmias occur in
15% and 25% of adults, respectively, and
require medical treatment
1. electrophysiologic study and ablation
2. defibrillator placement, or
3. transcatheter or surgical intervention
4. pulmonary valve replacement
Transcatheter pulmonary valve replacement
is widely used in patients meeting anatomic
criteria

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES

commonly called dextro- or D-transposition of the
great arteries
aorta arises rightward anteriorly from the RV, and
the pulmonary artery emerges leftward and posteriorly from the LV, which results in two separate parallel
circulations
Most patients have an interatrial communication
o two-thirds have a patent ductus arteriosus
o about one-third have an associated VSD
More common in males and accounts for ~10% of cyanotic heart disease
course is determined by:
1. the degree of tissue hypoxemia
2. the ability of each ventricle to sustain an increased workload in the presence of reduced coronary arterial
oxygenation
3. the nature of the associated cardiovascular anomalies
4. the status of the pulmonary vascular bed
Patients who do not undergo surgical palliation generally do not survive to reach adulthood
By the third decade of life, ~30% of patients with
atrial switch operations will have developed decreased RV function and progressive tricuspid regurgitation,
which may lead to congestive heart failure
Pulmonary vascular obstruction develops by 12 years
of age in patients with an associated large VSD or large patent ductus arteriosus in the absence of obstruction
to LV outflow
Treatme Transposition of the Great Arteries
nt
Balloon or blade catheter or surgical creation
or enlargement of an interatrial
communication in the neonate
simplest procedure for providing increased
intracardiac mixing of systemic and pulmonary
venous blood
Systemic pulmonary artery anastomosis may
be indicated in the patient with severe obstruction
to LV outflow and diminished pulmonary blood flow
Intracardiac repair
accomplished by rearranging the venous
returns (intraatrial switch, i.e., Mustard or
Senning operation)
the systemic venous blood is directed to the
mitral valve and, thence, to the LV and
pulmonary artery, while the pulmonary venous
blood is diverted through the tricuspid valve
and RV to the aorta
late survival after repairs is good
arrhythmias (e.g., atrial flutter) or conduction
defects (e.g., sick sinus syndrome) occur in
~50%
of patients by 30 years after the intraatrial
switch surgery

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SINGLE VENTRICLE
a family of complex lesions with both atrioventricular valves or a common atrioventricular valve opening to a single
ventricular chamber
Associated anomalies
o abnormal great artery positional relationships
o pulmonic valvular or subvalvular stenosis
o subaortic stenosis
Modifications of the Fontan approach are generally applied to carefully selected patients with creation of a pathway(s) from
the systemic veins to the pulmonary arteries
TRICUSPID ATRESIA
Characterized by atresia of the tricuspid valve; an interatrial communication; and, frequently, hypoplasia of the RV and
pulmonary artery
Severe cyanosis due to obligatory admixture of systemic and pulmonary venous blood in the LV
ECG: (1) RA enlargement, (2) left-axis deviation, and (3) LV hypertrophy
Atrial septostomy and palliative operations to increase pulmonary blood flow, often by anastomosis of a systemic
artery or vein to a pulmonary artery, may allow survival to the second or third decade
Fontan atriopulmonary or total cavopulmonary connection: allow functional correction in patients with normal or low
pulmonary arterial resistance pressure and good LV function
o Cosiderations:
1. development of arrhythmias
2. progressive liver dysfunction
3. thromboembolic complication
4. potential long-term need for heart or heart and liver transplantation
EBSTEINS ANOMALY
downward displacement of the tricuspid valve into the RV, due to anomalous attachment of the tricuspid leaflets, the Ebstein
tricuspid valve tissue is dysplastic and results in tricuspid regurgitation
Clinical manifestations are variable:
(1) progressive cyanosis from right-to-left atrial shunting
(2) symptoms due to tricuspid regurgitation and RV dysfunction
(3) paroxysmal atrial tachyarrhythmias with or without atrioventricular bypass tracts (Wolff-Parkinson-White [WPW]
syndrome)
Diagnostic Findings
o two-dimensional echocardiography: abnormal positional relation between the tricuspid and mitral valves with
abnormally increased apical displacement of the septal tricuspid leaflet
o Doppler examination: Tricuspid regurgitation is quantitated
Surgical approaches include prosthetic replacement of the tricuspid valve when the leaflets are tethered or repair of the
native valve
Two fundamental anatomic abnormalities
o Transposition of the ascending aorta and pulmonary trunk
o Inversion of the ventricles
This arrangement results in desaturated systemic venous
blood passing from the RA through the mitral valve to the LV
and into the pulmonary trunk, whereas oxygenated
pulmonary venous blood flows from the left atrium through
the tricuspid valve to the RV and into the aorta
Progressive RV dysfunction and tricuspid regurgitation may also develop in one-third of patients by age 30
VSD or PS due to obstruction to outflow from the right-sided subpulmonary (anatomic left) ventricle may coexist
Complete heart block occurs at a rate of 210% per
decade
Diagnosis of the malformation and associated lesions can
be established by comprehensive two-dimensional echocardiography and Doppler examination.
MALPOSITIONS OF THE HEART
Dextrocardia: is in the right side of the chest
Mesocardiac: cardiac apex is at the midline
Isolated levocardia: there is a normal location of the heart in the left side of the chest but abnormal position of the viscera
SURGICALLY MODIFIED CONGENITAL HEART DISEASE
Proper care of the survivor of an operation for CHD
Requires that the clinician understand the details of the malformation before operation
Pay meticulous attention to the details of the operative
procedure
Recognize the postoperative residua (conditions left
totally or partially uncorrected), the sequelae (conditions caused by surgery), and the complications that may have resulted
from the operation
Cardiac operations involving the atria: May be followed
a. closure of ASD years later by
b. repair of total or partial anomalous sinus node or
pulmonary venous return atrioventricular
c. venous switch corrections of node dysfunction
complete transposition of the great and/or by atrial
arteries (the Mustard or Senning arrhythmias
operations), (especially atrial
flutter)

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 Intraventricular surgery may also result in electrophysiologic consequences complete heart block necessitating pacemaker
insertion to avoid sudden death
After repair of the ostium primum ASD cleft mitral valve may become progressively regurgitant
Postoperative patient with tetralogy of Fallot Tricuspid regurgitation may also be progressive if RV outflow tract obstruction
was not relieved adequately at initial
surgery
Use of prosthetic valves, patches, or conduits in the operative repair
o Special risks: infective endocarditis, thrombus formation, and premature degeneration and calcification of the
prosthetic materials
The effect of pregnancy in postoperative patients depends on the outcome of the repair, including the presence and severity
of residua, sequelae, or complications
Tubal ligation should be considered in those in whom
pregnancy is strictly contraindicated
Endocarditis Prophylaxis
Two major predisposing causes:
1. cardiovascular substrate
2. source of bacteremia
Prophylaxis includes both antimicrobial and hygienic measures
Routine antimicrobial prophylaxis is recommended for bacteremic dental procedures or instrumentation through an infected site
in most patients with operated CHD, particularly if foreign material, such as a prosthetic valve, conduit, or surgically constructed
shunt, is in place
In the case of patches, in the absence of a high-pressure patch
leak, or transcatheter devices, prophylaxis is usually recommended for 6 months until there is endothelialization
Individuals with unrepaired cyanotic heart disease are also generally recommended to receive prophylaxis

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