Adult Congenital Heart Disease
Examination Questions And Correct
Answers (Verified Answers) Plus
Rationales 2026 Q&A | Instant
Download Pdf
1. A 32-year-old woman with a history of repaired tetralogy of Fallot presents
with progressive dyspnea on exertion and palpitations. Her transthoracic
echocardiogram demonstrates severe pulmonary regurgitation, a
moderately dilated right ventricle with a fractional area change of 30%, and
a mildly enlarged left ventricle with normal systolic function. The estimated
right ventricular systolic pressure is 25 mmHg. Which of the following is the
most appropriate next step in her management?
A. Initiate diuretic therapy to reduce right ventricular preload
B. Refer for cardiac magnetic resonance imaging to quantify regurgitant
fraction
C. Proceed directly to surgical pulmonary valve replacement
D. Schedule a transesophageal echocardiogram for further valve
assessment
Answer: B
*Rationale: The patient has significant pulmonary regurgitation with right
ventricular dilation and dysfunction. While echocardiography is the first-line
imaging tool, cardiac magnetic resonance (CMR) is the gold standard for
quantifying pulmonary regurgitant fraction and accurately measuring right
ventricular volumes and ejection fraction. These parameters are critical for
determining the optimal timing for pulmonary valve replacement. Surgery is
not indicated without a full quantification and symptom correlation.
, Diuretics are not primary therapy for this condition, and TEE is not superior
to CMR for volume quantification.
2. A 28-year-old man with a congenitally corrected transposition of the great
arteries (SLL) presents for routine follow-up. His systemic (morphologic
right) ventricle has a fractional shortening of 25% and an end-systolic
volume index of 60 mL/m². He is asymptomatic. What is the most
appropriate management strategy based on these findings?
A. Continue current observation as he is asymptomatic
B. Initiate an angiotensin-converting enzyme inhibitor
C. Refer for cardiac transplantation evaluation
D. Perform a double-switch operation
Answer: B
*Rationale: In congenitally corrected transposition, systemic right
ventricular dysfunction is a major predictor of adverse outcomes. Current
guidelines recommend initiation of afterload reduction therapy (e.g., ACE
inhibitors or angiotensin receptor blockers) for systemic right ventricular
dysfunction, even in asymptomatic patients, to slow the progression of
failure. The degree of dysfunction here is significant (fractional shortening <
30%). Observation alone is not appropriate. Transplantation is a later step,
and a double-switch is generally performed in childhood.
3. A 24-year-old woman with an unrepaired atrial septal defect (secundum
type) is evaluated for pregnancy. Her echocardiogram shows a left-to-right
shunt with a Qp:Qs ratio of 2.0:1 and a resting pulmonary artery pressure of
35 mmHg. The defect measures 18 mm with adequate rims. What is the
most appropriate recommendation regarding her care?
A. Proceed with pregnancy under close echocardiographic monitoring
B. Perform device closure prior to pregnancy
C. Close the defect surgically due to the size
D. Perform a right heart catheterization with vasoreactivity testing
Answer: D
*Rationale: Before pregnancy, it is essential to fully define the
hemodynamic significance of the shunt and rule out pulmonary
, hypertension. Right heart catheterization with vasoreactivity testing is the
gold standard to assess pulmonary pressures and calculate pulmonary
vascular resistance. If the pulmonary artery pressure is ≥ 35 mmHg, a
vasodilator trial is needed. Device closure is not recommended without
knowing the full hemodynamic picture, especially if reactive pulmonary
hypertension is present. Surgical closure is not first-line for this anatomy.
4. A 35-year-old male with a history of Fontan palliation (intra-atrial lateral
tunnel) presents with progressive cyanosis. An echocardiogram reveals a
large fenestration with right-to-left shunting. What is the most common
long-term complication associated with this fenestration that should be
monitored?
A. Systemic embolization
B. Paradoxical emboli
C. Protein-losing enteropathy
D. Hepatic congestion
Answer: A
*Rationale: While a fenestration may be beneficial in the early
postoperative period to offload the Fontan circulation, it can lead to right-
to-left shunting and cyanosis. The most feared long-term complication of
such a shunt is systemic embolization, as venous thrombi or air can bypass
the pulmonary filter and enter the systemic circulation, causing a stroke.
Paradoxical emboli is a mechanism, but the outcome is systemic
embolization. Protein-losing enteropathy and hepatic congestion are
complications of the Fontan physiology itself, not specifically the
fenestration.
5. A 30-year-old woman with Ebstein anomaly presents with progressive
palpitations. An electrocardiogram shows a wide complex tachycardia with
a left bundle branch block morphology and a superior axis. What is the
most likely arrhythmia mechanism?
A. Atrioventricular reentrant tachycardia (AVRT) using an accessory pathway
B. Atrioventricular nodal reentrant tachycardia (AVNRT)
C. Atrial fibrillation
, D. Ventricular tachycardia from the right ventricular outflow tract
Answer: A
*Rationale: Ebstein anomaly is strongly associated with accessory
pathways, most commonly right-sided (due to the displaced tricuspid valve
and atrialized right ventricle). The presence of a wide complex tachycardia
with LBBB morphology suggests a pathway that conducts antegradely, often
seen in pre-excitation. While atrial fibrillation and ventricular tachycardia
can occur, the classic association is AVRT. AVNRT typically presents with a
narrow complex tachycardia.
6. A 27-year-old man with a mechanical aortic valve (St. Jude Medical) in the
aortic position, placed for bicuspid aortic valve disease, is found to have a
peak instantaneous gradient of 45 mmHg and a mean gradient of 30 mmHg
on a routine follow-up echocardiogram. His left ventricular ejection fraction
is 60%. What is the most appropriate interpretation of these findings?
A. This is a normal gradient for a mechanical valve in this position
B. There is probable patient-prosthesis mismatch
C. There is significant prosthetic valve stenosis
D. This is consistent with a normally functioning valve
Answer: B
*Rationale: For a mechanical aortic valve, a mean gradient > 20 mmHg in a
patient with a normal LVEF is suggestive of patient-prosthesis mismatch
(PPM) or intrinsic stenosis. PPM occurs when the prosthetic valve is too
small for the patient’s body surface area, leading to high residual gradients.
A normal gradient is typically < 20 mmHg. Significant stenosis would be >
40-50 mmHg, but the clinical context and valve size should be evaluated.
This gradient is clearly above normal, and PPM is the most likely cause.
7. A 33-year-old female with a history of coarctation of the aorta, repaired at
age 5, presents with hypertension. Her echocardiogram shows a peak
gradient of 30 mmHg across the descending aorta with a continuous-wave
Doppler. What is the most accurate method to confirm the severity of this
re-coarctation?
A. Repeat transthoracic echocardiogram with color Doppler
Examination Questions And Correct
Answers (Verified Answers) Plus
Rationales 2026 Q&A | Instant
Download Pdf
1. A 32-year-old woman with a history of repaired tetralogy of Fallot presents
with progressive dyspnea on exertion and palpitations. Her transthoracic
echocardiogram demonstrates severe pulmonary regurgitation, a
moderately dilated right ventricle with a fractional area change of 30%, and
a mildly enlarged left ventricle with normal systolic function. The estimated
right ventricular systolic pressure is 25 mmHg. Which of the following is the
most appropriate next step in her management?
A. Initiate diuretic therapy to reduce right ventricular preload
B. Refer for cardiac magnetic resonance imaging to quantify regurgitant
fraction
C. Proceed directly to surgical pulmonary valve replacement
D. Schedule a transesophageal echocardiogram for further valve
assessment
Answer: B
*Rationale: The patient has significant pulmonary regurgitation with right
ventricular dilation and dysfunction. While echocardiography is the first-line
imaging tool, cardiac magnetic resonance (CMR) is the gold standard for
quantifying pulmonary regurgitant fraction and accurately measuring right
ventricular volumes and ejection fraction. These parameters are critical for
determining the optimal timing for pulmonary valve replacement. Surgery is
not indicated without a full quantification and symptom correlation.
, Diuretics are not primary therapy for this condition, and TEE is not superior
to CMR for volume quantification.
2. A 28-year-old man with a congenitally corrected transposition of the great
arteries (SLL) presents for routine follow-up. His systemic (morphologic
right) ventricle has a fractional shortening of 25% and an end-systolic
volume index of 60 mL/m². He is asymptomatic. What is the most
appropriate management strategy based on these findings?
A. Continue current observation as he is asymptomatic
B. Initiate an angiotensin-converting enzyme inhibitor
C. Refer for cardiac transplantation evaluation
D. Perform a double-switch operation
Answer: B
*Rationale: In congenitally corrected transposition, systemic right
ventricular dysfunction is a major predictor of adverse outcomes. Current
guidelines recommend initiation of afterload reduction therapy (e.g., ACE
inhibitors or angiotensin receptor blockers) for systemic right ventricular
dysfunction, even in asymptomatic patients, to slow the progression of
failure. The degree of dysfunction here is significant (fractional shortening <
30%). Observation alone is not appropriate. Transplantation is a later step,
and a double-switch is generally performed in childhood.
3. A 24-year-old woman with an unrepaired atrial septal defect (secundum
type) is evaluated for pregnancy. Her echocardiogram shows a left-to-right
shunt with a Qp:Qs ratio of 2.0:1 and a resting pulmonary artery pressure of
35 mmHg. The defect measures 18 mm with adequate rims. What is the
most appropriate recommendation regarding her care?
A. Proceed with pregnancy under close echocardiographic monitoring
B. Perform device closure prior to pregnancy
C. Close the defect surgically due to the size
D. Perform a right heart catheterization with vasoreactivity testing
Answer: D
*Rationale: Before pregnancy, it is essential to fully define the
hemodynamic significance of the shunt and rule out pulmonary
, hypertension. Right heart catheterization with vasoreactivity testing is the
gold standard to assess pulmonary pressures and calculate pulmonary
vascular resistance. If the pulmonary artery pressure is ≥ 35 mmHg, a
vasodilator trial is needed. Device closure is not recommended without
knowing the full hemodynamic picture, especially if reactive pulmonary
hypertension is present. Surgical closure is not first-line for this anatomy.
4. A 35-year-old male with a history of Fontan palliation (intra-atrial lateral
tunnel) presents with progressive cyanosis. An echocardiogram reveals a
large fenestration with right-to-left shunting. What is the most common
long-term complication associated with this fenestration that should be
monitored?
A. Systemic embolization
B. Paradoxical emboli
C. Protein-losing enteropathy
D. Hepatic congestion
Answer: A
*Rationale: While a fenestration may be beneficial in the early
postoperative period to offload the Fontan circulation, it can lead to right-
to-left shunting and cyanosis. The most feared long-term complication of
such a shunt is systemic embolization, as venous thrombi or air can bypass
the pulmonary filter and enter the systemic circulation, causing a stroke.
Paradoxical emboli is a mechanism, but the outcome is systemic
embolization. Protein-losing enteropathy and hepatic congestion are
complications of the Fontan physiology itself, not specifically the
fenestration.
5. A 30-year-old woman with Ebstein anomaly presents with progressive
palpitations. An electrocardiogram shows a wide complex tachycardia with
a left bundle branch block morphology and a superior axis. What is the
most likely arrhythmia mechanism?
A. Atrioventricular reentrant tachycardia (AVRT) using an accessory pathway
B. Atrioventricular nodal reentrant tachycardia (AVNRT)
C. Atrial fibrillation
, D. Ventricular tachycardia from the right ventricular outflow tract
Answer: A
*Rationale: Ebstein anomaly is strongly associated with accessory
pathways, most commonly right-sided (due to the displaced tricuspid valve
and atrialized right ventricle). The presence of a wide complex tachycardia
with LBBB morphology suggests a pathway that conducts antegradely, often
seen in pre-excitation. While atrial fibrillation and ventricular tachycardia
can occur, the classic association is AVRT. AVNRT typically presents with a
narrow complex tachycardia.
6. A 27-year-old man with a mechanical aortic valve (St. Jude Medical) in the
aortic position, placed for bicuspid aortic valve disease, is found to have a
peak instantaneous gradient of 45 mmHg and a mean gradient of 30 mmHg
on a routine follow-up echocardiogram. His left ventricular ejection fraction
is 60%. What is the most appropriate interpretation of these findings?
A. This is a normal gradient for a mechanical valve in this position
B. There is probable patient-prosthesis mismatch
C. There is significant prosthetic valve stenosis
D. This is consistent with a normally functioning valve
Answer: B
*Rationale: For a mechanical aortic valve, a mean gradient > 20 mmHg in a
patient with a normal LVEF is suggestive of patient-prosthesis mismatch
(PPM) or intrinsic stenosis. PPM occurs when the prosthetic valve is too
small for the patient’s body surface area, leading to high residual gradients.
A normal gradient is typically < 20 mmHg. Significant stenosis would be >
40-50 mmHg, but the clinical context and valve size should be evaluated.
This gradient is clearly above normal, and PPM is the most likely cause.
7. A 33-year-old female with a history of coarctation of the aorta, repaired at
age 5, presents with hypertension. Her echocardiogram shows a peak
gradient of 30 mmHg across the descending aorta with a continuous-wave
Doppler. What is the most accurate method to confirm the severity of this
re-coarctation?
A. Repeat transthoracic echocardiogram with color Doppler
