Cardiac Embryology

Question 1

Simple Adult Congenital Heart Disease– Native Disease

Answer 1

Uncomplicated congenital aortic valve disease
Mild congenital mitral valve disease (e.g., except parachute valve, cleft leaflet)
Uncomplicated small atrial septal defect
Uncomplicated small ventricular septal defect
Mild pulmonic stenosis

Question 2

Simple Adult Congenital Heart Disease– Repaired Conditions

Answer 2

Previously ligated or occluded ductus arteriosus
Repaired secundum or sinus venosus atrial septal defect without residua
Repaired ventricular septal defect without residual

Question 3

Intermediate Complexity Congenital Heart Disease (11)

Answer 3

Ostium primum or sinus venosus atrial septal defect
Anomalous pulmonary venous drainage, partial or total
Atrioventricular canal defects (partial or complete)
Ventricular septal defect, complicated (e.g., absent or abnormal valves or with associated obstructive lesions, aortic regurgitation)
Coarctation of the aorta
Pulmonic valve stenosis (moderate to severe)
Infundibular right ventricular outflow obstruction of significance
Pulmonary valve regurgitation (moderate to severe)
Patent ductus arteriosus (non-closed)—moderate to large
Sinus of Valsalva fistula/aneurysm
Subvalvular or supravalvular aortic stenosis

Question 4

Complex Adult Congenital Heart Disease (9)

Answer 4

Cyanotic congenital heart diseases (all forms)
Eisenmenger’s syndrome
Ebstein’s anomaly
Tetralogy of Fallot or pulmonary atresia (all forms)
Transposition of the great arteries
Single ventricle; tricuspid or mitral atresia
Double-outlet ventricle
Truncus arteriosus
Fontan or Rastelli procedures

Question 5

Sinus venosus Atrial Septal Defect (ASD)

Answer 5

High in the atrial septum near the entry of the superior vena cava into the right atrium
Frequently associated with anomalous pulmonary venous connection from the right lung to the superior vena cava or right atrium.

Question 6

Ostium primum ASDs

Answer 6

Lie adjacent to the atrioventricular valves which may be deformed/regurgitant
Common in Down syndrome; may be more complex with a common atrioventricular valve (endocardial cushion defect)

Question 7

Ostium secundum ASD

Answer 7

Involves the fossa ovalis and is midseptal (as opposed to a patent foramen ovale)
“Probe patency” is common & trivial (functionally closed)
ASD denotes a true deficiency of the atrial septum and implies functional and anatomic patency

Question 8

Symptoms of ASD in Adults (6)

Answer 8

Usually asymptomatic in early life (may have growth retardation, increased URIs)

Past the 4th decade of life (especially ostium primum)
Atrial arrhythmias
Pulmonary arterial hypertension
Bidirectional and then right-to-left shunting of blood
Right heart failure.

In older patients, L to R shunting leads to progressive systemic hypertension and/or coronary artery disease (CAD) from reduced compliance of the LV

Chronic environmental hypoxemia of high altitude leads to pulmonary hypertension at younger ages

Question 9

ASD Physical Exam in Adults (8)

Answer 9

Prominent RV impulse and palpable pulmonary artery pulsation

1st heart sound is normal or split, with accentuation of the tricuspid valve closure sound

Midsystolic pulmonary outflow murmur due increased flow across pulmonic valve

2nd heart sound is widely split and is relatively fixed in relation to respiration

Mid-diastolic rumbling murmur (4th L intercostal space) reflects increased flow across the tricuspid valve

Ostium primum ASD with apical holosystolic murmur (usually mitral or tricuspid regurgitation or a ventricular septal defect (VSD)

Cyanosis and clubbing accompany the development of a right-to-left shunt

Increased pulmonary resistance leads to diminished L to R shunt
Both pulmonary outflow and tricuspid inflow murmurs decrease in intensity
Pulmonic component of the 2nd heart sound and a systolic ejection sound are accentuated

Question 10

ASD Echo (3)

Answer 10

Pulmonary arterial and RV and RA dilatation
Abnormal (paradoxical) ventricular septal motion in the presence of a significant right heart volume overload
ASD may be visualized directly by two-dimensional imaging, color-flow imaging, or echocontrast.

Question 11

ASD Treatment- Operative Repair

Answer 11

Patch of pericardium or of prosthetic material or percutaneous transcatheter device closure
all patients with uncomplicated secundum ASD with significant left-to-right shunting, i.e., pulmonary-to-systemic flow ratios 1.5:1 - 2:1
In the absence of pulmonary HTN, excellent results even over 40 yo
Closure should not be carried out in patients with small defects and trivial left-to-right shunts or in those with severe pulmonary vascular disease without a significant left-to-right shunt

Question 12

ASD Treatment- Medical Management

Answer 12

(morbidity increases in patents > 40 yo)
Prompt treatment of respiratory-tract infections
Anti-arrhythmic medications for atrial fibrillation or supraventricular tachycardia
Rx of hypertension, coronary disease, or heart failure

Question 13

Ventricular Septal Defect (VSD)

Answer 13

Most common of all cardiac birth defects
Isolated defects
Component of a combination of anomalies
Usually single and situated in the membranous or mid-muscular portion of the septum

Question 14

VSD in Adults (6)

Answer 14

Dysfunction depends on its size and on the status of the pulmonary vascular bed

Only small- or moderate-size VSDs are seen initially in adulthood

Most patients with an isolated large VSD come to medical or surgical attention early in life

Spontaneous closure is more common in patients born with a small VSD (infancy & early childhood in most)

Pulmonary vascular bed determines clinical manifestations and feasibility of surgical repair

Increased pulmonary arterial pressure results from increased pulmonary blood flow and/or resistance leading to obstructive, obliterative structural changes within the pulmonary vascular bed

Question 15

Eisenmenger’s syndrome

Answer 15

Large communication between the two circulations at the aortopulmonary, ventricular or atrial levels

Bidirectional or predominantly right-to-left shunts because of high resistance and obstructive pulmonary hypertension

Large VSDs should be corrected surgically early in life when pulmonary vascular disease is still reversible or not yet developed

Symptoms in adults with Eisenmenger (exertional dyspnea, chest pain, syncope, and hemoptysis)

Right-to-left shunt leads to cyanosis, clubbing, and erythrocytosis

Question 16

Surgical Outcomes for Eisenmenger’s syndrome

Answer 16

Pulmonary vascular resistance < 1/3 of systemic – good post surgical prognosis, no progression of pulmonary vascular disease
Moderate to severe pulmonary vascular resistance exists preoperatively, either no change or a progression of pulmonary vascular disease is common postoperatively

Question 17

Additional Complications of VSD

Answer 17

RV outflow tract obstruction in 5–10% of patients who present in infancy with a moderate to large left-to-right shunt.
As subvalvular RV outflow tract obstruction progresses, patients with large VSDs develop a hemodynamic pattern resembling cyanotic tetralogy of Fallot.
5% - aortic valve regurgitation results from insufficient cusp tissue or prolapse of the cusp through the interventricular defect; the aortic regurgitation then complicates and dominates the clinical course

Question 18

Presentation of VSD in Adults (5)

Answer 18

Asymptomatic murmur may not be present at birth, develop in the 1st few months as the VSD constricts and disappear
Restrictive VSD, holosystolic murmer with normal S2 in 12 yo
Muscular VSD in 6 yo, short systolic not extending to S2 (small defect closes as muscle contracts)
Loud short systolic VSD murmur in infant, single S2 c/w pulmonary HTN
Pulmonary-to-systemic flow increases leading to severe pulmonary hypertension
Eisenmenger’s syndrome

Question 19

Still’s Murmur

Answer 19

Vibratory quality
Location: left midsternal border

Confused with:
VSD
subpulmonic stenosis

Question 20

Venous Hum

Answer 20

Continuous
Usually loudest when sitting
Location: neck and under clavicles

Confused with:
Patent ductus arteriosus
Coronary AV malfunction

Question 21

Systolic Ejection Murmur at the Base of the Heart

Answer 21

High left sternal border
Confused with: pulm stenosis, ASD
High Right Sternal Border— confused with aortic stenosis

Question 22

Surgery Treatment for VSD

Answer 22

Surgery is not recommended for patients with normal pulmonary arterial pressures with small shunts (pulmonary-to-systemic flow ratios of 1.5:1 or 2:1
Absence of prohibitively high levels of pulmonary vascular resistance
Pregnancy is contraindicated due to pulmonary vascular HTN, mother is also at risk for arrythmias, fetus at risk with maternal cyanosis, CHF

Question 23

Erythrocytosis VSD Treatment

Answer 23

Chronic hypoxemia in cyanotic CHD results in secondary erythrocytosis due to increased erythropoietin production
Rx not indicated in compensated erythocytosis, even with Hct of 65-70%
Decompensated erythrocytosis with unstable, rising Hct causing hyperviscosity syndorme and warrants therapeutic phebotomy
Phebolomy relieves hyperviscoity symptoms but limits O2 delivery and adds to iron depletion

Question 24

Iron Deficiency Treatment for VSD

Answer 24

Hypochromic microcytosis. less capable of carrying O2 and less deformable
Viscosity becomes a problem with more RBCs per plasma volume which are less deformable

Question 25

Patent Ductus Arteriosus

Answer 25

Ductus arteriosus is a vessel leading from the bifurcation of the pulmonary artery to the aorta just distal to the left subclavian artery

Normally, open in the fetus but closes immediately after birth (O2 tension, bradykinins)

Flow across a patent ductus is determined by the pressure and resistance relationships between the systemic and pulmonary circulations and by the cross-sectional area and length of the ductus

Question 26

Poiseuille’s Equation

Answer 26

vessel resistance (R) is directly proportional to the length (L) of the vessel and theviscosity(η) of the fluid/air, and inversely proportional to the radius to the fourth power (r4)

Question 27

Clinical Presentation in most adults with PDA

Answer 27

Pulmonary pressures are normal
A gradient and shunt from aorta to pulmonary artery persist throughout the cardiac cycle
Characteristic thrill and a continuous “machinery” murmur with late systolic accentuation at the upper left sternal edge

Question 28

Adults born with a large left-to-right PDA shunt Clinical Presentation

Answer 28

Pulmonary vascular obstruction (Eisenmenger syndrome) with pulmonary hypertension
Right-to-left shunting
Cyanosis from unoxygenated blood shunting to the descending aorta
Toes—but not the fingers—become cyanotic and clubbed, a finding termed differential cyanosis.

Question 29

Leading causes of death in adults with PDA

Answer 29

Cardiac failure
Infective endocarditis
Pulmonary vascular obstruction may cause aneurysmal dilatation, calcification, and rupture of the ductus

Question 30

Patent Ductus ArteriosusTreatment - Adults

Answer 30

Closure recommended in adults without severe pulmonary vascular disease & R to L shunt
Surgical ligation and division
Transcatheter closure using coils, buttons, plugs, and umbrellas has become commonplace for appropriately shaped defects
Thoracoscopic surgical approaches are considered experimental
Closure deferred for several months after infective endocarditis because the ductus may remain edematous and friable

Question 31

Aortic Root–to-Right-Heart Shunts — Congenital aneurysm of an aortic sinus of Valsalva with fistula

Answer 31

Separation or lack of fusion between the media of the aorta and the annulus of the aortic valve
Rupture usually occurs 3rd or 4th decade of life
Most often fistula between R coronary cusp and R ventricle
Occasionally fistula is between non-coronary cusp and R atrium
Abrupt rupture causes chest pain, bounding pulses, continuous murmur accentuated in diastole, volume overload of the heart
Medical management is directed at cardiac failure, arrhythmias, or endocarditis

Question 32

Diagnosis of Aortic Root–to-Right-Heart Shunts — Congenital aneurysm of an aortic sinus of Valsalva with fistula

Answer 32

Two-dimensional and Doppler echocardiographic studies
Cardiac catheterization quantitates the left-to-right shunt
Thoracic aortography visualizes the fistula

Question 33

Surgical Treatment for Aortic Root–to-Right-Heart Shunts — Congenital aneurysm of an aortic sinus of Valsalva with fistula

Answer 33

aneurysm is closed and amputated and the aortic wall is reunited with the heart, either by direct suture or patch/prosthesis

Question 34

Aortic Root–to-Right-Heart Shunts Coronary arteriovenous fistula

Answer 34

Unusual anomaly - communication between a coronary artery and another cardiac chamber, usually the coronary sinus, right atrium, or right ventricle
Shunt is usually of small magnitude and myocardial blood flow is not usually compromised;
Shunt large - coronary “steal” syndrome with myocardial ischemia and possible angina or ventricular arrhythmias

Question 35

Complications of Aortic Root–to-Right-Heart Shunts Coronary arteriovenous fistula

Answer 35

Infective endocarditis
Thrombus formation with occlusion or distal embolization with myocardial infarction
Rupture of an aneurysmal fistula
Rarely - pulmonary hypertension and congestive failure

Question 36

Doppler Echo, Angiography of Aortic Root–to-Right-Heart Shunts Coronary arteriovenous fistula

Answer 36

Loud, superficial, continuous murmur at the lower or midsternal border usually prompts a further evaluation of asymptomatic patients.
Doppler echocardiography demonstrates the site of drainage; if the site of origin is proximal, it may be detectable by two-dimensional echocardiography
Angiography (classic catheterization, CT, or magnetic resonance angiography) permits identification of the size and anatomic features of the fistulous tract, which may be closed by suture or transcatheter obliteration

Question 37

Aortic Root–to-Right-Heart Shunts Anomalous origin of the left coronary artery from the pulmonary artery

Answer 37

Myocardial infarction and fibrosis commonly lead to death within the first year
20% of patients survive to adolescence and beyond without surgical correction
Diagnosis - ECG findings of an anterolateral MI & left ventricular hypertrophy (LVH)
Surgery - CABG (with internal mammary artery graft or saphenous vein)

Question 38

Congenital Aortic Stenosis– Bicuspid Aortic Valve

Answer 38

One of the most common congenital heart malformations, not always detectable
Murmur harsh systolic
More common in males than in females
May initially be functionally normal
Bicuspid valves may develop stenosis or regurgitation with time or be the site of infective endocarditis

Question 39

Valvular Aortic StenosisTreatment

Answer 39

Medical
? Prophylaxis against infective endocarditis ?
Patients with diminished cardiac reserve, the administration of digoxin and diuretics and sodium restriction while awaiting operation.
Dilated aortic root may require beta blockers.
Surgical – aortic valve replacement
Critical obstruction (aortic valve area <0.45 cm2/m2
Symptoms 2nd secondary LV dysfunction or myocardial ischemia
Hemodynamic evidence of LV dysfunction
Aortic balloon valvuloplasty
Asymptomatic children/adolescent/young adults with critical aortic stenosis without calcifications
When surgery contraindicated balloon valvuloplasty may provide short-term or bridge to value replacement with CHF

Question 40

Subaortic Stenosis

Answer 40

Discrete form of subaortic stenosis consists of a membranous diaphragm or fibromuscular ring encircling the LV outflow tract just beneath the base of the aortic valve
Jet impact from the subaortic stenotic jet on the underside of the aortic valve leads to progressive aortic valve fibrosis and valvular regurgitation
Echocardiography demonstrates the anatomy of the subaortic obstruction; Doppler studies show turbulence proximal to the aortic valve; quantitate the pressure gradient and severity of aortic regurgitation
Treatment - complete excision of the membrane/fibromuscular ring

Question 41

Supravalvular Aortic Stenosis

Answer 41

Localized or diffuse narrowing of the ascending aorta just above the level of the coronary arteries at the superior margin of the sinuses of Valsalva
Coronary arteries are subjected to elevated systolic pressures, often dilated, tortuous, atherosclerotic
Most patients have a genetic defect for the anomaly (located in the same chromosomal region as elastin on chromosome 7

Question 42

Coarctation of the Aorta

Answer 42

Narrowing or constriction of the lumen of the aorta, most often distal to the origin of the left subclavian artery near the insertion of the ligamentum arteriosum
Occurs in 7% of patients with congenital heart disease, is more common in males than females, and is particularly frequent in patients with gonadal dysgenesis (e.g., Turner syndrome)

Question 43

Clinical Manifestation for Coarctation of the Aorta

Answer 43

Site
Extent of obstruction
Associated cardiac anomalies; bicuspid aortic valve (up to 75% association)
Circle of Willis aneurysms may occur in up to 10%, high risk of sudden rupture and death

Question 44

ECG and CXR for Coarctation of the Aorta

Answer 44

ECG - may show LV hypertrophy
CXR - may show dilated left subclavian artery high on the left mediastinal border, 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 - essentially pathognomonic

Question 45

Presentation of Coarctation of the Aorta

Answer 45

Most children/young adults with isolated, discrete coarctation are asymptomatic
Headache, epistaxis, cold extremities, and claudication with exercise may occur
1st findings may be heart murmur and HTN in upper extremities; low or delayed pulses in femoral arteries on PE
Enlarged and pulsatile collateral vessels may be palpated in the intercostal spaces anteriorly, in the axillae, or posteriorly in the interscapular area
Upper extremities and thorax may be more developed than the lower extremities
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

Question 46

Coarctation of the Aorta Treatment

Answer 46

Surgical resection and reanastomosis
Percutaneous catheter balloon dilatation with stent placement
Late postoperative systemic hypertension in the absence of residual coarctation is related partly to the duration of preoperative hypertension
Follow-up of rest and exercise blood pressures is important; many have systolic hypertension only during exercise, in part due to a diffuse vasculopathy.

Question 47

Pulmonary Stenosis with Intact Ventricular Septum

Answer 47

RV outflow obstruction localized to the supravalvular, valvular, or subvalvular levels or occur in any combination
Multiple peripheral sites of narrowing seen in rubella embryopathy & familial and sporadic forms of supravalvular aortic stenosis
Valvular pulmonic stenosis (PS) is the most common form of isolated RV obstruction

Question 48

Pulmonary Stenosis with Intact Ventricular Septum Severity Ranges

Answer 48

Severity of the obstructing lesion, rather than the site of narrowing, main determinant of the clinical course and symptoms
Mild PS - peak systolic pressure gradient 50 mmHg indicates severe PS – severity may progress
Moderate - 30-50 mmHg - fatigue, dyspnea, RV failure, and syncope may limit the activity of older patients, where cardiac output may not increase with exertion

Question 49

Pulmonary Stenosis with Intact Ventricular Septum– Severe Obstruction

Answer 49

Systolic pressure in the RV may exceed that in the LV, because the ventricular septum is intact
RV ejection is prolonged with moderate or severe stenosis
Sound of pulmonary valve closure is delayed and soft
RV hypertrophy reduces the compliance of that chamber
Forceful RA contraction is necessary to augment RV filling
4th heart sound
Prominent a waves in the jugular venous pulse
Presystolic pulsations of the liver reflect vigorous atrial contraction

Question 50

Pulmonary Stenosis Workup

Answer 50

ECG
Mild PS – normal
Severe PS – RV hypertrophy
CXR
Mild or moderate PS – normal heart size and pulmonary vascularity
Valvular stenosis – dilatation of main and left pulmonary arteries (2nd to jet and intrinsic media weakness)
Severe obstruction – RV hypertrophy with reduced pulmonary vessels
Two-dimensional echocardiography visualizes pulmonary-valve morphology; the outflow tract pressure gradient is quantitated by Doppler echocardiography

Question 51

Pulmonary Stenosis - Treatment

Answer 51

Cardiac catheter technique of balloon valvuloplasty is usually effective
Direct surgical relief of moderate and severe obstruction may be accomplished at a low risk
Multiple stenoses of the peripheral pulmonary arteries are usually inoperable
Some proximal branch or bifurcation lesions of the main PA may be surgically corrected or undergo balloon dilatation and stenting

Question 52

4 Components of Tetralogy of Fallot

Answer 52

Malaligned VSD
RV outflow obstruction
Overriding aortic (over VSD)
RV hypertrophy due to the RV’s response to aortic pressure via the large VSD

Question 53

Clinical Presentation of TOF

Answer 53

Severity of RV outflow obstruction determines the clinical presentation
Hypoplasia of the RV outflow tract varies from mild to complete (pulmonary atresia)
Pulmonary valve stenosis and supravalvular and peripheral pulmonary arterial obstruction may coexist
Rarely, there is unilateral absence of a pulmonary artery (usually left)
Right-sided aortic arch and descending thoracic aorta occur in 25%

Question 54

Hemodynamics of TOF

Answer 54

Determined by relationship between the resistance of blood flow from the ventricles into the aorta and into the pulmonary artery
When the RV outflow obstruction is severe, pulmonary blood flow is reduced markedly, and a large volume of desaturated systemic venous blood shunts right-to-left across the VSD
Severe cyanosis and erythrocytosis occur
In many infants and children, the obstruction is mild but progressive

Question 55

TOF Treatment

Answer 55

Tet Spell
RV infundibular contractility 
Catecholamine release
Hyperventilation w/ increased WOB
R to L shunt

Treatment
Increase SVR
Increase R filling pressure
Reduce catecholamine release
Supplemental O2

Question 56

ECG and CXR for TOF

Answer 56

ECG
RV hypertrophy

CXR
Normal-sized, boot-shaped heart (coeur ‘heart’ en sabot ‘wooden shoe worn in some European countries’)
Prominent right ventricle
Concavity in the region of the pulmonary conus
Pulmonary vascular markings are typically diminished
Aortic arch and knob may be on the right side (25%)
Two-dimensional echocardiography
Malaligned VSD with the overriding aorta and variable site and severity of PS

Question 57

TOF Treatment

Answer 57

Balloon or blade catheter or surgical creation interatrial communication in the neonate increases 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

Question 58

Long Term Complications of TOF

Answer 58

Only a few adults with tetralogy of Fallot have not had some form of previous surgical intervention
Reoperation in adults is most commonly for severe pulmonary regurgitation
Long-term problems with ventricular function
Ventricular and atrial arrhythmias occur, requiring medical treatment, electrophysiologic study & ablation
Interventional catheterization may be needed in selected patients (i.e., angioplasty and stenting of branch pulmonary stenosis)
Aortic root has a medial tissue defect; it is commonly enlarged and associated with aortic regurgitation
Endocarditis remains a risk despite surgical repair

Question 59

Complete Transposition of the Great Arteries

Answer 59

Dextro- or d-transposition of the great arteries
Aorta arises rightward anteriorly from the RV
Pulmonary artery emerges leftward and posteriorly from the LV
Results in two separate parallel circulations (some communication between them must exist after birth to sustain life)
Most have intra-atrial communication
2/3 have PDA
1/3 have associated VSD .

Question 60

Epidemiology of Complete Transposition of the Great Arteries

Answer 60

Transposition is more common in males
10% of cyanotic heart disease
Clinical course influenced by
Degree of tissue hypoxemia
Ability of each ventricle to sustain an increased workload in the presence of reduced coronary arterial oxygenation
Nature of associated cardiovascular anomalies
Status of the pulmonary vascular bed
By 30 yo, 30% of patients will have developed decreased RV function and progressive tricuspid regurgitation and CHF
Pulmonary vascular obstruction develops by one to two years of age in patients with an associated large VSD or large PDA in the absence of obstruction to LV outflow

Question 61

Transposition of the Great Arteries Intra-atrial Switch

Answer 61

Intracardiac repair by rearranging the venous returns (intra-atrial switch, i.e., Mustard operation)
Systemic venous blood is directed to the mitral valve then LV then PA
Pulmonary venous blood is diverted through the tricuspid valve and right ventricle to the aorta
Late survival after these repairs is good
Arrhythmias (e.g., atrial flutter) or conduction defects (e.g., sick sinus syndrome) occur in 50% of such patients by 30 years after the intra-atrial switch surgery
Progressive dysfunction of the systemic subaortic right ventricle with risk of tricuspid regurgitation, ventricular arrhythmias, or cardiac arrest and late sudden death

Question 62

Transposition of the Great Arteries Rastelli Procedure

Answer 62

Patients with VSD with severely obstructed LV outflow tract
Operation uses an intracardiac ventricular baffle and extracardiac prosthetic conduit to replace the pulmonary artery (Rastelli procedure)

Question 63

Congenitally Corrected Transposition

Answer 63

Two fundamental anatomic abnormalities
Transposition of the ascending aorta and pulmonary trunk
Inversion of the ventricles.
Desaturated systemic venous blood passing from the right atrium through the mitral valve to the LV and into the pulmonary trunk,
Oxygenated pulmonary venous blood flows from the left atrium through the tricuspid valve to the RV and into the aorta.
Circulation is corrected functionally

Question 64

Results of Congenitally Corrected Transposition

Answer 64

Clinical presentation and prognosis determined by:
Nature and severity of any complicating intracardiac anomalies
Development of dysfunction of the systemic subaortic RV
1/3 of patients by 30 yo develop progressive RV dysfunction and tricuspid regurgitation
Ebstein-type anomalies of the left-side tricuspid atrioventricular valve are common
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 2–10% per decade

Question 65

Total Anomalous Venous Return

Answer 65

Total anomalous pulmonary venous connection (showing pulmonary veins connected to the left innominate vein)
superior vena cava
atrial septal defect
left innominate vein
pulmonary veins
Oxygenated blood returning from the lungs is routed back into the superior vena cava, rather than the left atrium
Presence of an atrial septal defect is necessary to allow partially oxygenated blood to reach the left side of the heart

Question 66

Partial Anomalous Pulmonary Venous Return

Answer 66

Partial anomalous pulmonary venous drainage
location where right pulmonary veins normally enter left atrium
right pulmonary veins entering right atrium
Oxygenated blood flows from the right pulmonary veins into the right atrium instead of into the left atrium

Question 67

Tricuspid Atresia

Answer 67

Associated lesions:
Interatrial communication;
Commonly, hypoplasia of the right ventricle and pulmonary artery
Clinical presentation includes severe cyanosis due to obligatory admixture of systemic and pulmonary venous blood in the left ventricle
ECG characteristically shows RA enlargement, left-axis deviation, and LV hypertrophy

Question 68

Ebstein’s Anomaly

Answer 68

Downward displacement of the tricuspid valve into the right ventricle
Anomalous attachment of the tricuspid leaflets
Tricuspid valve tissue is dysplastic
Tricuspid regurgitation
“Atrialized” portion of the RV lying between the atrioventricular ring and the origin of the valve, which is continuous with the RA chamber
RV is often hypoplastic

Question 69

Ebstein’s Anomaly Clinical Presentation

Answer 69

Progressive cyanosis from right-to-left atrial shunting
Symptoms due to tricuspid regurgitation and RV dysfunction
Paroxysmal atrial tachyarrhythmias with or without atrioventricular bypass tracts [Wolff-Parkinson-White (WPW) syndrome

Question 70

CXR and Echo for Ebstein’s Anomaly

Answer 70

CXR – cardiomegaly, RA enlargement, decreased pulmonary vascularity
Two-dimensional echocardiography shows abnormal positional relation between the tricuspid and mitral valves with abnormally increased apical displacement of the septal tricuspid leaflet
Tricuspid regurgitation is quantitated by Doppler examination
Surgery with prosthetic replacement of the tricuspid valve when the leaflets are tethered or repair of the native valve

Question 71

Kartagener‘s Syndrome

Answer 71

CXR
Dextrocardia
Hyperinflation of the lungs
Linear tram-track opacities suggestive of bronchiectasis
Kartagener syndrome (part of ciliary dyskinesia / immobile cilia syndrome)
Autosomal recessive disease with extensive genetic heterogeneity
Abnormal ciliary motion
Impaired mucociliary clearance
Situs inversus

Question 72

Clinical Presentation of Kartagener’s Syndrome

Answer 72

complete situs inversus (situs inversus totalis)
chronic sinusitis
bronchiectasis
telecanthus : widened interpupillary distance by a nasal polyp
azoospermia
Radiographic changes are much milder than in cystic fibrosis

Question 73

Malpositions of the Heart

Answer 73

Positional anomaly - cardiac apex
Right side of the chest (dextrocardia)
Midline (mesocardia)
Left side of the chest but abnormal position of the viscera (isolated levocardia)
Mirror-image dextrocardia with complete situs inversus usually has normal cardiac findings otherwise
Malposition of the abdominal organs relative to cardiac multiple cardiac anomalies are usually present

Question 74

Ectopia Cordis

Answer 74

Ectopia cordis, ectocardia or exocardia, is a rare congenital defect, resulting in herniation of the heart through a sternal, pericardial and/or abdominal defect
Defect in the fusion of the anterior chest wall
Pentalogy of Cantrell
defects in diaphragm
midline supra-umbilical abdominal wall defect
pericardium hear
lower sternum
congenital intracardiac abnormalities

Question 75

Single Ventricle

Answer 75

Single ventricular chamber with complex or common atrioventricular valves
Associated anomalies
Abnormal great artery positional relationships
Pulmonic valvular or subvalvular stenosis
Subaortic stenosis
Survival to adulthood depends on a relatively normal pulmonary blood flow with normal pulmonary resistance and good ventricular function.
Modifications of the Fontan approach with systemic to pulmonary artery anastomoses

Question 76

Surgically Modified Congenital Heart Disease

Answer 76

Except for ligation of an uncomplicated patent ductus arteriosus, almost every other surgical repair leaves behind or causes some abnormality of the heart and circulation that may range from trivial to serious

Cardiac operations importantly involving the atria, such as closure of ASD, repair of total or partial anomalous pulmonary venous return, or venous switch corrections of complete transposition of the great arteries (the Mustard or Senning operations), may be followed years later by sinus node or atrioventricular node dysfunction or by atrial arrhythmias (especially atrial flutter)

Intraventricular surgery may also result in electrophysiologic consequences, including complete heart block necessitating pacemaker insertion to avoid sudden death

Valvular problems may arise late after initial cardiac operation
Progressive stenosis of an initially nonobstructive bicuspid aortic valve in the patient who underwent aortic coarctation repair
Cleft mitral valve may become progressively regurgitant after repair of the ostium primum ASD
Tricuspid regurgitation may be progressive in the postoperative patient with tetralogy of Fallot (when RV outflow tract obstruction was not relieved with initial surgery)
Following surgically modified CHD, inadequate relief of an obstructive lesion, residual regurgitant lesion or shunt will lead to myocardial dysfunction
Patients who were cyanotic for many years before repair, have a preexisting compromise in ventricular performance

Question 77

Antibiotic prophylaxis is indicated for the following high-risk cardiac conditions:

Answer 77

Prosthetic cardiac valve
History of infective endocarditis
Congenital heart disease (CHD) (except for the conditions listed, antibiotic prophylaxis is no longer recommended for any other form of CHD): (1) unrepaired cyanotic CHD, including palliative shunts and conduits; (2) completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure; and (3) repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibits endothelialization)
Cardiac transplantation recipients with cardiac valvular disease