Congenital Heart Disease Flashcards
The view of choice when examining a patient with secundum atrial septal defect is:
A. Apical four-chamber
B. Parasternal long-axis
C. Parasternal short-axis of the aortic valve
D. Subcostal four-chamber
D. The subcostal four-chamber view places the inter-atrial septum perpendicular to the ultrasound beam which is the optimal way to interrogate the inter-atrial septum since it is a specular reflector. The parasternal short-axis view and the apical four chamber view with color flow Doppler on or agitated saline contrast added may also be useful.
A defect is found in the central portion of the inter-atrial septum. The type of atrial septal defect present is:
A. Ostium secundum
B. Ostium primum
C. Sinus venosus
D. Coronary sinus
A. The most common type of atrial septal defect (70%) is the ostium secundum type which is located in the mid-portion of the inter-atrial septum.
Ostium primum atrial septal defect is the second most common type (15%) and is located in the lower part of the inter-atrial septum and is strongly associated with cleft mitral valve (partial atrioventricular septal defect).
Sinus venous atrial septal defect is located in the upper portion of the inter-atrial septum and is strongly associated with partial anomalous pulmonary venous return (PAPVR).
All of the following are associated echocardiographic findings for atrial septal defect EXCEPT:
A. Right atrial enlargement
B. Right ventricular enlargement
C. Left ventricular enlargement
D. Paradoxical interventricular septal motion
C. Atrial septal defect is considered to be a right heart volume overload resulting in right atrial enlargement, right ventricular enlargement and paradoxical interventricular septal motion. The combination of right ventricular enlargement and paradoxical interventricular septal motion is called the right ventricular volume overload pattern.
Tricuspid regurgitation is an expected cardiac Doppler finding due to right ventricular enlargement (functional tricuspid regurgitation) which will allow the calculation of the right ventricular systolic pressure and systolic pulmonary artery pressure.
The normal Qp/Qs ratio is:
A. 1:1
B. 2:1
C. 1:2
D. 50%
A. Qp/Qs is way to quantitate the size of a shunt lesion (e.g., atrial septal defect, ventricular septal defect, patent ductus arteriosus). Normally the stroke volume of the right ventricle and left ventricle are equal so the normal Qp/Qs is 1:1. A significant shunt is a Qp/Qs of 2:1.
The stroke volume for each ventricle is required. Measurement of the right ventricular and left ventricular outflow tracts diameter during early ventricular systole and the velocity time integrals for each ventricle measured with pulsed-wave Doppler at the level of the outflow tracts is required to determine Qp/Qs.
When evaluating atrial septal defect flow with color Doppler, the sonographer should:
A. Invert the color flow map
B. Reduce the color velocity scale
C. Invert the color flow map
D. Increase the color Doppler filter
B. Atrial septal defect flow is usually low velocity flow (< 1 m/s). By decreasing the color flow velocity scale, the pulse repetition frequency (PRF) is reduced which will allow lower velocity flow to be detected.
For an agitated saline contrast exam, where will contrast appear proving the patient has an atrial septal defect?
A. Right atrium
B. Left atrium
C. Right ventricle
D. Main pulmonary artery
B. In the presence of an atrial septal defect contrast material will cross the inter-atrial septum and enter into the left atrium and left ventricle.
Additionally, a negative contrast effect (darkened area in the right atrium due to blood shunting from the left atrium to the right atrium) may be noted. For ventricular septal defect the negative contrast effect would be seen in the right ventricle.
A potential complication of patent foramen ovale is:
A. Paradoxical embolus
B. Mitral valve stenosis
C. Valvular stenosis
D. Congestive heart failure
A. Patent foramen ovale (PFO) may be present in up to 30% of adults and represents a potential site for paradoxical embolism especially if right heart pressures are elevated. An agitated saline contrast study may be required to determine the presence of a PFO. To improve the detection of PFO during an agitated saline contrast study the Valsalva maneuver (release phase) or cough may be required.
A redundancy of the mid-portion of the atrial septum which may result in an inter-atrial shunt is called:
A. ASA
B. ASH
C. DSS
D. SAM
A. Atrial septal aneurysm (ASA) is redundancy of the mid-portion of the atrial septum with increased motion of that region (> 10 mm from the plane of the inter-atrial septum). Atrial septal aneurysm is a potential location for shunting and a careful color flow Doppler exam or agitated saline contrast exam is required. The Valsalva maneuver or cough may be required to induce/enhance the shunt.
In addition, thrombi may form in the pouches of the atrial septal aneurysm which may result in thromboembolism.
The most common type of ventricular septal defect is:
A. Perimembranous
B. Trabecular
C. Inlet
D. Outlet (supracristal)
A. Membranous defects are the most common type (80%) of ventricular septal defect and are located beneath the right and noncoronary aortic valve leaflets. The parasternal long-axis, parasternal short-axis at the cardiac base, apical five-chamber and apical long-axis views with color flow Doppler on are useful two-dimensional views.
Aortic regurgitation is a common Doppler finding in perimembranous (as well as outlet) ventricular septal defect.
The type of ventricular septal defect most often associated with ventricular septal aneurysm is:
A. Perimembranous
B. Trabecular
C. Inlet
D. Outlet
A. Tricuspid valve tissue may overlay the perimembranous ventricular septal defect creating a ventricular septal aneurysm. The aneurysm is often seen as a thin membranous pouch and should be evaluated with Doppler to determine the presence of a residual shunt.
The cardiac chambers that are enlarged in ventricular septal defect initially are:
A. Left atrium; left ventricle
B. Right atrium; left atrium
C. Right atrium; right ventricle
D. Right ventricle; left ventricle
A. The left atrium and left ventricle will be increased in dimension because of increased flow through the lungs. Eventually the increased flow volume in the lungs will increase the pulmonary vascular resistance resulting in a right ventricular pressure overload and potentially Eisenmenger’s syndrome.
In a patient with ventricular septal defect the blood pressure is 120/80 mm Hg and the peak systolic velocity of the ventricular septal defect is 5 m/s. The right ventricular systolic pressure and systolic pulmonary artery pressure is:
A. 120 mm Hg
B. 110 mm hg
C. 30 mm Hg
D. 20 mm Hg
D. Estimation of the right ventricular systolic pressure (RVSP) and systolic pulmonary artery pressure (SPAP) using continuous-wave Doppler and applying the formula:
RVSP and SPAP (mm Hg) = SBP - 4 x VSD peak velocity^2
For this question:
RVSP and SPAP (mm Hg) = 120 mm Hg - 4 x 5^2 m/s
120 mm Hg - 100 mm Hg = 20 mm Hg
SBP, systolic blood pressure;
VSD, ventricular septal defect
A complete atrioventricular septal defect is ostium primum atrial septal defect with:
A. Coarctation of the aorta
B. Cleft mitral valve
C. Canal (inlet)-type ventricular septal defect, patent ductus arteriosus
D. Canal (inlet)-type ventricular septal defect, common atrioventricular valve
D. Endocardial cushion defects (also referred to as atrioventricular canal defects, atrioventricular septal defects) may be partial, complete or incomplete. An ostium primum atrial septal defect with cleft mitral valve is a partial atrioventricular septal defect.
The congenital heart defect most often associated with Down syndrome (trisomy 21) is:
A. Coarctation of the aorta
B. Atrioventricular septal defect
C. Peripheral pulmonary stenosis
D. Tetralogy of Fallot
B. The two most common types of cardiac lesions associated with Down’s syndrome are complete atrioventricular septal defect and ventricular septal defect.
A congenital malformation of the tricuspid valve in which one, two or all three leaflets are displaced downward from the annulus with right ventricular dysplasia (atrialization) is known as:
A. Ebstein’s anomaly
B. Epstein-Barr anomaly
C. Tricuspid atresia
D. Tricuspid stenosis
A. Ebstein’s anomaly consists of apical displacement of the tricuspid valve leaflets with dysplasia of the right ventricle. The two-dimensional view to evaluate Ebstein’s anomaly is the apical four-chamber view. A displacement of ≥ 20 mm or 8 mm^2 as measured from the mitral valve annulus to the tricuspid valve displacement indicates Ebstein’s anomaly. Tricuspid regurgitation and functional tricuspid stenosis are expected cardiac Doppler findings.
Congenital heart defects strongly associated with Ebstein’s anomaly include:
A. Atrial septal defect
B. Coarctation of the aorta
C. Discrete subaortic stenosis
D. Parachute mitral valve
A. An inter-atrial communication (e.g., ostium secundum atrial septal defect, patent foramen ovale) is present in most cases (80%) which may result in a right-to-left shunt causing cyanosis. Pulmonary atresia, pulmonary stenosis, ventricular septal defect, mitral valve prolapse and poor right ventricular and left ventricular global systolic function may be associated complications.
Uhl’s anomaly is:
A. Left atrial aneurysm
B. Abnormal displacement of the tricuspid valve
C. Right ventricular dysplasia
D. Ostium primum atrial septal defect with cleft mitral valve
C. Uhl’s anomaly (parchment heart) is a congenital disorder where the right ventricular myocardium is absent and is replaced with fibro-fatty tissue. It is the most extreme form of right ventricular dysplasia. The echocardiography findings include a very thin walled right ventricle (1 to 2 mm in thickness), markedly reduced global and segmental right ventricular systolic function and low velocity (< 2 m/s) tricuspid regurgitation.
Patent ductus arteriosus results in:
A. Right ventricular volume overload
B. Left ventricular volume overload
C. Right ventricular pressure overload
D. Left ventricular pressure overload
B. The left-to-right shunt of a patent ductus arteriosus (PDA) results in volume overload of the left ventricle and left atrium due to the increased flow to the lungs. Eventually the increased pulmonary flow may result in an increase in pulmonary pressures and a right ventricular pressure overload and Eisenmenger’ syndrome.
The typical murmur associated with patent ductus arteriosus is:
A. Decrescendo diastolic murmur
B. Continuous murmur
C. Holosystolic murmur
D. Late systolic murmur
B. Because aortic pressure is greater than pulmonary artery pressure throughout the cardiac cycle the murmur of patent ductus arteriosus is a continuous, noisy, machinery-like murmur.
Other causes of continuous murmur includes ruptured sinus of Valsalva aneurysm, jugular venous hum and coronary arteriovenous fistula.
The peak velocity across a patent ductus arteriosus is 4 m/s and the blood pressure is 90/60 mm Hg. The systolic pulmonary artery pressure is:
A. 4 mm Hg
B. 26 mm Hg
C. 36 mm Hg
D. 64 mm Hg
B. In patent ductus arteriosus, the SPAP (mm Hg) is calculated by the formula:
Systolic blood pressure (mm Hg) - 4 x Vmax PDA^2
For this question: SPAP mm Hg = 90 - 64 = 26 mm Hg.
SPAP, systolic pulmonary artery pressure;
Vmax, maximum velocity;
PDA, patent ductus arteriosus
The Doppler finding associated with patent ductus arteriosus is:
A. Holodiastolic flow reversal in the descending thoracic aorta
B. Increased flow velocity at the aortic isthmus
C. Decreased pressure half-time of the mitral valve
D. Systolic flow reversal in the pulmonary veins
A. Other causes of holodiastolic flow reversal in the descending thoracic aorta include severe aortic regurgitation, ruptured sinus of Valsalva aneurysm, aortopulmonary window and cerebrovascular arteriovenous fistula.
A communication between the ascending aorta and the main pulmonary artery is called:
A. Aortopulmonary window
B. Coarctation of the aorta
C. Patent ductus arteriosus
D. Supracristal ventricular septal defect
A. The anatomic defect is similar to the pathophysiology of patent ductus arteriosus.
Narrowing of the aortic isthmus is:
A. Aortic arch aneurysm
B. Aortic dissection
C. Coarctation of the aorta
D. Patent ductus arteriosus
C. Coarctation of the aorta is narrowing of the aorta at or near the site of the insertion of the ductus arteriosus. Types of coarctation of the aorta include preductal, juxtaductal and postductal.
In coarctation of the aorta blood pressure in the legs:
A. Is lower than the right arm
B. Is higher than in the right arm
C. Is equal to blood pressure in the right arm
D. Cannot be compared with blood pressure in the right arm
A. Blood pressure is usually lower in the legs than in the arms in aortic coarctation unless collateral flow is well developed. Blood pressure in the left arm can be affected by the location of a coarctation and so is not a reliable indicator of upper body blood pressure. In patients six or older impingement of collaterals on the ribs causes rib notching on chest-x-ray.
Valvular lesions with which coarctation of the aorta is strongly associated include:
A. Aortic valve flail
B. Aortic valve vegetation
C. Bicuspid aortic valve
D. Tricuspid atresia
C. Coarctation of the aorta is frequently associated with bicuspid aortic valve (50 to 80%). Other anomalies include patent ductus arteriosus, ventricular septal defect and mitral valve abnormalities.
The continuous-wave Doppler tracing shown below is most likely:
A. Mitral regurgitation
B. Aortic regurgitation
C. Pulmonary stenosis
D. Coarctation of the aorta
D. The classic continuous-wave Doppler finding for coarctation of the aorta is increased peak systolic and end-diastolic velocities (“sawtooth” appearance).
The suprasternal long-axis of the aorta is the view of choice for the evaluation of aortic coarctation.
All of the following are associated with pulmonary stenosis EXCEPT:
A. Right ventricular hypertrophy
B. Systolic doming of the pulmonary valve
C. Pulmonary regurgitation
D. Coarctation of the aorta
D. Additionally post-stenotic dilatation of the main pulmonary artery may be present and is more common in mild cases of pulmonary stenosis.
The four defects that make up tetralogy of Fallot are right ventricular outflow tract obstruction (e.g., pulmonary stenosis), ventricular septal defect, right ventricular hypertrophy and:
A. Atrial septal defect
B. Deviation of the aorta
C. Cleft mitral valve
D. Coarctation of the aorta
B. In 1888 Etienne Louis Arthur Fallot reported a malformation that consists of the following: (1) stenosis of the pulmonary artery, (2) interventricular communication, (3) deviation of the origin of the aorta to the right and (4) hypertrophy, almost always concentric, of the right ventricle.
Prime characteristics of tetralogy of Fallot include all of the following EXCEPT:
A. Atrial septal defect
B. Malalignment ventricular septal defect
C. Right ventricular outflow tract obstruction
D. Right ventricular hypertrophy
A. Tetralogy of Fallot is characterized by right ventricular outflow tract obstruction, overriding of the aorta in relation to the interventricular septum, right ventricular hypertrophy and malalignment ventricular septal defect.
A right-sided aortic arch is present in 30% of tetralogy of Fallot cases.
Defects associated with tetralogy of Fallot in aproximately 30% of cases include:
A. Bicuspid aortic valve
B. Overriding pulmonary artery
C. Tricuspid atresia
D. Right aortic arch
D. In addition, coronary artery anomalies should be carefully evaluated in patients with tetralogy of Fallot. A coronary artery branch crossing the right ventricular outflow tract such as a left anterior descending coronary artery or conus branch has important surgical implications.
Mitral valve abnormalities such as double orifice mitral valve should also be sought in patients with tetralogy of Fallot.
Important factors in evaluating post-surgical repair of tetralogy of Fallot include all of the following EXCEPT:
A. Evaluate right and left ventricular function
B. Rule out residual right ventricular outflow tract obstruction
C. Rule out residual shunting at the margins of the atrial septal defect repair
D. Rule out shunting at the margins of the ventricular septal defect repair
C. In addition the presence and severity of pulmonary regurgitation should be assessed.
Eisenmenger’s syndrome may be associated with all of the following EXCEPT:
A. Atrial septal defect
B. Bicuspid aortic valve
C. Ventricular septal defect
D. Patent ductus arteriosus
B. Eisenmenger’s syndrome is the reversal of any congenital shunt from left-to-right to right-to-left secondary to pulmonary hypertension.
Eisenmenger’s complex is defined as a cyanotic heart defect consisting of ventricular septal defect, dextroposition of the aorta, pulmonary hypertension and right ventricular hypertrophy.
The physical finding of cyanosis is most common in:
A. Patent foramen ovale
B. Eisenmenger’s syndrome
C. Mitral valve prolapse
D. Pulmonary regurgitation
B. Eisenmenger’s syndrome is the reversal of long-standing left-to-right intracardiac shunt with right ventricular and pulmonary artery systolic pressures equaling or exceeding systemic pressure.
All of the following surgical repairs for congenital heart disease are correctly matched EXCEPT:
A. Modified Glenn: Superior vena cava to the right pulmonary artery
B. Blalock-Taussig: Right subclavian artery to the right pulmonary artery
C. Fontan: Single ventricle repair
D. Ross: Surgical ligation of a patent ductus arteriosus
D. The Ross procedure is the placement of the native pulmonary valve in the aortic valve position with re-implantation of the coronary arteries and placement of a pulmonary homograft in the pulmonary position.
