Self-Assessment Examination

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Aggressive monitoring for the development of PAH should occur in Fontan patients because:

1. They often have a mean pulmonary pressure >25 mm Hg at right heart catherization

2. An Eisenmenger physiology is associated in most cases

3. A conduit obstruction may lead to cardiac decompensation

4. Even slight increase in PVR may have significant hemodynamic consequences

Which of the following forms of congenital heart disease is most likely to lead to the development of PAH?

1. Partial anomalous pulmonary venous return without repair

2. Secundum atrial septal defect without repair

3. An unrestricted ventricular septal defect without prior repair

4. Patent foramen ovale without repair

A 30-year-old woman is diagnosed with a membranous VSD. She undergoes echocardiographic imaging evaluation and an invasive hemodynamic study. The hemodynamic study demonstrates pulmonary artery pressures of 110/50 mm Hg. Her central aortic pressure is 100/60 mm Hg. A pulmonary venous oximetry sample has a saturation of 95% and her femoral artery oximetry sample has a saturation of 86%. There is severe pulmonary regurgitation, as well. Which of the following is correct regarding treatment?

1. Closure of the VSD is indicated

2. PAH vasodilator therapy only is indicated

3. Heart transplantation should be considered

4. She should have pulmonary valve replacement

Which of the following is the most common subtype of ASD?

1. Muscular

2. Outlet

3. Secundum

4. Sinus venosus

5. Membranous

Echocardiographic indicators of poor outcomes in patients with Eisenmenger syndrome are:

1. Pericardial effusion + low TAPSE

2. Low TAPSE + shortened RV filling time + increased RA area/LA area

3. Pericardial effusion + low TAPSE + shortened RV filling time

4. Bi-atrial enlargement

In Eisenmenger patients, the presence of RV late gadolinium enhancement at cardiac MRI:

1. Is a pathologic finding in any cases

2. Is usually located at the apex

3. Is a normal feature typically evident at the insertion points

4. When present at the insertion points is an indicator of poor outcome

Catheter-based interventions are available for all of the following lesions except:

1. Secundum atrial septal defect

2. Muscular ventricular septal defect

3. Ostium primum atrial septal defect

4. Patent ductus arteriosus

In a 4-month-old infant with APAH-CHD due to a nonrestrictive VSD, what would you most expect hemodynamics to resemble on cardiac catheterization?

1. Elevated pulmonary artery pressure, normal wedge pressure, elevated pulmonary blood flow, normal pulmonary vascular resistance

2. Elevated pulmonary artery pressure, elevated wedge pressure, normal pulmonary blood flow, normal pulmonary vascular resistance

3. Normal pulmonary artery pressure, normal wedge pressure, normal pulmonary blood flow, normal pulmonary vascular resistance

4. Elevated pulmonary artery pressure, normal wedge pressure, normal pulmonary blood flow, elevated pulmonary vascular resistance

The BREATHE-5 trial involving which endothelin receptor antagonist was the first randomized, double-blind, placebo-controlled drug trail conducted solely on Eisenmenger patients?

1. Sildenafil

2. Bosentan

3. Ambrisentan

4. Treprostinil