1. (B) For asymptomatic patients with secundum ASDs, right heart enlargement, and no pulmonary hypertension, there is a 2A recommendation to proceed with ASD closure (compared to a grade 1 recommendation for those who are symptomatic). Her right heart is moderately enlarged and RVSP is normal. Therefore, there is no need for invasive Q_p/Q_s measurement as there is no concern for pulmonary hypertension.

2. (A) The 2018 AHA/ACC ACHD guidelines varied from the original 2008 statement. The 2008 guidelines stated that pulmonary valve replacement for repaired tetralogy of Fallot was only recommended if the pulmonary regurgitation was severe and had any of the following symptoms: ≥moderate RV enlargement or dysfunction, ≥moderate tricuspid regurgitation, or symptomatic arrhythmias. The 2018 guidelines state that in asymptomatic individuals (Physiological Stage A) with ≥moderate pulmonary regurgitation, pulmonary valve replacement is reasonable if there are any two of the following: mild or moderate RV or LV systolic dysfunction, severe RV dilation (RVEDVI ≥160 mL/m², RVESVI ≥80 mL/m², or RVEDV/LVEDV >2.0), RVSP ≥2/3 systemic pressure, or progressive reduction in exercise tolerance. As this patient has moderate-severe pulmonary regurgitation and a severely enlarged RV with moderate systolic dysfunction, pulmonary valve replacement would be reasonable at this time.

3. (E) This patient with repaired tetralogy of Fallot and moderate pulmonary regurgitation has progression of symptoms and is now NYHA Class III. The 2018 AHA/ACC guidelines recommend that for symptomatic patients with ≥moderate pulmonary regurgitation, pulmonary valve replacement is reasonable. Results of MRI, exercise testing, or repeat echocardiogram would not outweigh her symptoms and heart failure. Although tricuspid regurgitation may improve after percutaneous pulmonary valve implantation, given the severity of the TR and the tricuspid valve dilation, surgery may be more effective for this patient. However, when answering a question similar to this on the boards realize that guideline recommendations (hence the correct answer on the test) and clinical practice for the individual patient may differ regarding choice of catheter versus surgical intervention.

4. (A) According to the 2018 AHA/ACC guidelines, ICD implantation would be reasonable for primary prophylaxis in a patient with tetralogy of Fallot given numerous risk factors (LV dysfunction, nonsustained VT, QRS duration ≥180 ms, and extensive RV scarring). While proceeding with electrophysiology study and potential ablation is reasonable, these are not necessary prior to ICD implantation.

5. (D) According to the AHA/ACC 2018 guidelines, pulmonary vasoactive medications are reasonable to use in patients with Fontan physiology for improvement in exercise tolerance. He has a borderline increased transpulmonary gradient of 8 mm Hg, suggestive of pulmonary vascular disease in a patient after Fontan. PDE5 inhibitors such as sildenafil and endothelin antagonists have been utilized in this patient population. There are limited data regarding prostacyclin analogues (e.g., epoprostenol); and furthermore, this medication requires continuous intravenous infusion, thus it would not be preferred over oral sildenafil therapy. Oxygen therapy is unlikely to improve his saturation significantly given his known venovenous collaterals. Fontan revision is mostly recommended when there are refractory atrial tachyarrhythmias. While transplant referral may be reasonable, medical therapy is a more appropriate next step

6. (D) According to the 2018 AHA/ACC guidelines, this patient is IA with a simple congenital abnormality and is Physiological Stage A with no symptoms. Physical examination and echocardiogram are reassuring that this is a small, restrictive VSD with no left heart enlargement or pulmonary hypertension. There is no significant associated valve disease. Thus, she likely will never need closure of this VSD due to the shunt. But routine follow-up is recommended to assess progression of aortic valve regurgitation. Aortic valve cusp prolapse may occur in patients with membranous VSDs. The guidelines do not have recommendations for use of cardiac MRI in long-term follow-up of VSDs in adults.

7. (B) According to the AHA/ACC Valve guidelines, surgery is indicated for asymptomatic individuals with severe aortic regurgitation if there is significant LV enlargement (LVESV >50 mm, LVESVi >25 mm/m²) or dysfunction (LV ejection fraction <55%). In addition, in patients with bicuspid aortic valves, aortic dilation >55 mm in those without risk factors (family history, rapid growth, or coarctation) or >50 mm in those with risk factors, is an indication for ascending aorta and valve replacement.

8. (D) Sotalol is a Class III antiarrhythmic, blocking the IKr channel, thus it delays ventricular repolarization and can prolong the QT interval. Excessive prolongation of the QT interval can lead to polymorphic VT (torsades de pointes). This is more likely to occur with hypokalemia, hypomagnesemia, and bradycardia. The type of anticoagulant does not make torsades more likely. The 2018 AHA/ACC guidelines recommend anticoagulation with a vitamin K antagonist (i.e., warfarin) in all patients after Fontan with atrial arrhythmias and no contraindications to anticoagulation. However, novel agents are currently under investigation for this purpose also.

9. (C) This patient with prior surgical repair of tetralogy of Fallot has now developed right-sided heart failure from constrictive pericarditis and severe pulmonary regurgitation. This is suggested by his elevated jugular venous pressure and the contour with prominent descents classic for constrictive physiology. In addition, he has a pericardial knock. The lateral film shows calcification of the diaphragmatic pericardium. Further evaluations with echocardiography, MRI, and/or catheterization are important prior to surgical referral. Percutaneous pulmonary valve replacement would not address the pericardial disease. He has newly diagnosed heart failure, so recommending routine follow-up or exercise without further assessment is not appropriate.

10. (B) There are multiple complications of the Ross procedure including pulmonary homograft degeneration, neo-aortic valve degeneration, aortic root dilation, and coronary ostial narrowing/occlusion. This patient had degeneration of his pulmonary homograft requiring placement of a conduit and this has now degenerated. He has symptomatic RV-PA conduit obstruction and warrants intervention. Given the loud and palpable P₂, this is unlikely to purely be valvular stenosis and rather there is sub- or supravalvular stenosis as well. Given the multilevel obstruction, he will need either surgical replacement of the entire conduit or percutaneous valve replacement with stenting of the conduit. CT imaging specifically to evaluate the extra-anatomic conduit and the course of the coronary arteries relative to the conduit will be essential for either percutaneous or surgical planning. In this situation, CT imaging is superior to MR.

11. (C) The shunt is exclusively left to right and there is RV enlargement. Therefore, the shunt is large. In this scenario, PVR is likely low enough that it can be argued that calculation of pulmonary pressures and resistance at cardiac cath are not essential. She will require some form of coronary artery imaging given her age >40 to 45 years. Life expectancy with a large ASD is reduced in adults >40 years, with arrhythmia and elevated PA pressures. Treating her solely with device closure will not be sufficient to address the Afib. Surgical closure with Maze is likely the best course. This would also be true if there was severe TR associated with the ASD. Tricuspid valve repair could be addressed at time of surgical ASD closure.

12. (A) One of the earliest signs of cirrhotic liver disease from any etiology is thrombocytopenia. It is present in at least 70% of patients with cirrhosis. The pathophysiology is due to splenic sequestration, decreased production of thrombopoietin (TPO), and decreased bone marrow production. A platelet count <100,000 is a marker for advanced liver disease and increased mortality. Elevation of biomarkers that detect synthetic dysfunction appear late in FALD. Elevation of the INR, in a patient not receiving a vitamin K antagonist, may be an earlier manifestation of synthetic liver dysfunction. Alpha fetoprotein is a marker for hepatocellular carcinoma.

13. (C) Objectively assessing functional capacity and VO₂ can be very helpful during the assessment of adults with CHD. Arrhythmias that may prompt earlier intervention may be provoked with exercise. In an adult with ToF and severe residual PR, most would recommend PVR if there are overt symptoms, new arrhythmia, RVEDVi >150 mL/m², RV/LV >2:1, > mild TR, > mild RV systolic dysfunction, or any LV dysfunction.

14. (C) During a normal pregnancy, the following physiologic changes occur: blood volume increases by 40%, systemic and pulmonary vascular resistance decrease, and heart rate increases. This all leads to a 30% increase in cardiac output. Blood pressure is the least affected by pregnancy.

15. (E) The image shows a bifid uvula. The patient has peripheral aneurysms. She likely has Loeys-Dietz. These patients have increased risk of aortic dissection when the aortic root is >40 mm. Surgical intervention is recommended in Loeys-Dietz patients at 40 mm. In this patient, valve-sparing root replacement may not be possible if there already is significant aortic valve regurgitation. Prior to reaching 40 mm, aggressive medical management with ARBs is indicated for all patients with Loeys-Dietz.

16. (C) Review the modified Duke criteria (see below). A and B are minor criteria, D (changing degree of regurgitation) does not satisfy a major criterion, E—both culture bottles should be positive to satisfy a major criterion.

Major criteria:

17. (A) Intranasal cocaine use is a risk for HCV infection. The patients described in (B) and (D) had blood transfusions, but after 1992 and neither were born before 1965. The patient described in C has no risk factors.

The following need screening for hepatitis C infection:

18. (C) She needs to start warfarin therapy to avoid valve thrombosis. Subcutaneous heparin is an inferior choice for therapeutic anticoagulation long term and IV heparin long term is not practical. LMWH can be used, but needs weekly anti-Xa monitoring, not daily. NOAC/DOACs have not been approved for use in pregnancy.

19. (A) Patients with bileaflet mechanical aortic valve replacements and no other risk factors for thrombosis do not need bridging.

20. (C) Cyanosis is an important risk factor for maternal and fetal complications. While baseline exercise intolerance and NYHA class II symptoms are important to note, cyanosis is a more important risk factor. Bioprosthetic valves with normal function confer less risk than mechanical valves.

21. (A) Microcytosis caused by iron deficiency in patients with secondary erythrocytosis creates the greatest risk for thromboembolic events. Phlebotomy for these patients is rarely performed in the current era. It is reserved for patients with neurologic symptoms or prior to surgery. Hydration is also a successful therapy for these patients. Thrombocytopenia and mild leukopenia are characteristic of patients with unrepaired cyanotic CHD.

22. (D) Women with Eisenmenger syndrome are at exceptionally high risk of complication and death during pregnancy and the peripartum period. Pregnancy should be avoided in these patients. The patient described in answer A has one predictor of cardiac complications (predicted risk 25%) but has a low risk of death. Patients with Marfan syndrome can have dissection during pregnancy, but pregnancy is not absolutely contraindicated unless the aorta is >40 mm. The patient described in answer C has a risk of cardiac complications, but no absolute contraindication.

23. (A) The arterial switch operation for d-TGA requires reimplantation of coronary artery buttons. There is risk of both early and late coronary obstruction. There is evidence that the risk may be greater with single coronary artery anatomy. Symptoms of coronary ischemia may present in atypical fashion. The history of exertional chest pain that resolved with rest warrants evaluation. Coronary angiography is the gold standard for coronary assessment, although computed tomography angiography may be appropriate.

24. (D) Mustard and Senning atrial switch procedures are associated with sinus node dysfunction and atrial arrhythmias. Other complications are baffle leaks and baffle obstruction. Placement of transvenous, intracardiac pacemaker leads may worsen baffle stenosis, and paradoxical embolus may occur across a baffle leak. Incorrect positioning of ventricular leads across the baffles is occasionally observed. Pacemaker placement in this patient should be performed at a center with experience in adult congenital cardiac care. There is no history given for thrombophilia. While sudden death does occur in patients with repaired d-TGA, there is no history of atrial or ventricular tachycardia in this patient even with exercise.

25. (B) Isolated membranous VSD is associated with the development of DCRV that may occur in adulthood. DCRV is defined by a proximal portion of the RV that is at high pressure, separated by abnormal muscular hypertrophy from a distal low-pressure zone. The pulmonary arterial pressure is distal to the obstruction and should be normal. The development of DCRV is often heralded by an increase in murmur intensity, onset of a thrill, and findings of RV pressure loading including an increased right ventricular impulse and RVH by ECG. Surgical resection of the muscle bundle is needed.

26. (B) The 2018 ACHD guidelines concluded that at this time there is insufficient evidence to recommend medications, such as ACE inhibitors or beta blockers, for patients with failing systemic right ventricles. His pulmonary hypertension is likely postcapillary in nature and pulmonary vasodilators would worsen his symptoms. His AV valve regurgitation is only moderate and likely due to ventricular dilation; furthermore, his ventricle may not tolerate AV valve replacement. There is no evidence to suggest obstruction of the systemic venous baffles given only mild jugular venous distention, no hepatomegaly, and patent baffles demonstrated on echocardiogram.

27. (D) The patient has a bileaflet mechanical aortic valve which functions normally. There is a relatively low likelihood of thrombosis with a brief period of holding anticoagulation. She has no additional risk factors for thrombosis (e.g., prior venous thromboembolism, atrial fibrillation, or solid organ malignancy), it is appropriate to hold warfarin 5 days prior to surgery, continue aspirin, and no bridging is needed. Alternatively, if she had risk factors for thrombosis, malfunctioning aortic prosthesis, or if the mechanical prosthesis was in another position, then bridging with a heparin product would be recommended.

28. (C) This young woman has never had alternative imaging of her aorta. Since she has dilation of the ascending aorta, it would be appropriate to perform more extensive imaging at least once early in adulthood and prior to pregnancy. She has mild aortic valve stenosis and regurgitation and likely would tolerate pregnancy well. Unless obstetrical complications occur, one would expect a normal spontaneous vaginal delivery. ACE inhibitors and angiotensin receptor blockers are contraindicated during pregnancy.

29. (B) It is considered safe to continue Warfarin at a dose of 5 mg or less daily if an INR of 3.0 can be obtained during pregnancy. The risk of warfarin embryopathy at a dose of less than 5 mg daily is felt to be low enough that one could continue Warfarin during pregnancy. Alternatively, if one cannot maintain an INR of 3.0 on this dose and need a higher dose, then Warfarin should be discontinued by the 6th week of gestation and resumed after the 12th week of gestation. Low-molecular-weight heparin 1 mg/kg twice daily should be administered. The patient should have her Anti-Xa level checked weekly or twice weekly depending on the clinical situation. Anti-Xa levels of 0.7 to 1.3 should be obtained. If this is a mechanical AV valve, one would want to have a higher Anti-Xa level. Warfarin should be discontinued at 36-week gestation in anticipation of delivery. Patients should be switched to low-molecular-weight heparin at this time. The last dose of low-molecular-weight heparin should be 12 hours prior to planned delivery. Postpartum if there is no unexpected bleeding, warfarin therapy can be reinstituted while bridging with low-molecular-weight heparin until the INR is at least 2.0. Pregnancy is considered a hypercoagulable state due to the elevated estrogen levels. Patients remain in this state of hypercoagulability in the first several weeks postpartum.

30. (C) The woman likely has Holt-Oram syndrome (radial hypoplasia associated with secundum atrial septal defect). It is inherited on an autosomal dominant fashion; therefore 50% of her offspring would be expected to inherit this syndrome assuming complete penetrance.

31. (B) See Answer 30. In patients with Holt-Oram syndrome, 75% have CHD.

32. (D) This case scenario demonstrates a typical presentation of a young adult with a previously undiagnosed ASD. Transthoracic imaging prior to intervention demonstrates a centrally located defect that is amenable to device closure. This defect can also be closed surgically. If the pulmonary veins were adequately visualized entering the left atrium, then no further imaging is required. Her clinical examination is consistent with a large left-to-right shunt from an atrial level defect (presence of diastolic rumble). There is no evidence on echocardiography or clinical examination for pulmonary hypertension, therefore cardiac catheterization would not be needed.

33. (C) The diastolic flow rumble in a patient with an ASD is due to excess flow across a normal tricuspid valve. Presence of this sound is associated with a Q_p/Q_s >1.5 to 2.0. The systolic murmur, similarly, is due to excess flow across an otherwise normal pulmonary valve. Flow across the ASD is of low velocity and inaudible. Young children with large VSDs will have a diastolic flow murmur due to flow across the mitral valve. Diastolic murmurs are not “normal.”

34. (C) The 2013 ACC/AHA guidelines recommend either high intensity or moderate intensity statin regimen for patients with an ASCVD risk score that is ≥7.5%. Gemfibrozil and Niacin are not statins. Aspirin therapy is not contraindicated but would not address this issue directly. Losartan does not treat hyperlipidemia

35. (C) The patient has a residual right-to-left shunt and atrial arrhythmia with spontaneous echo contrast in the right atrium. Risk of paradoxical embolus is important. Based on the ACHD guidelines, warfarin therapy is indicated.

36. (E) The fenestration gradient is equal to the transpulmonary gradient (RA or Fontan pressure minus LA pressure). Values of 5 to 8 mm Hg usually are expected. An increased gradient correlates with obstruction in the Fontan circuit, lungs or pulmonary veins. It is primarily dependent on total pulmonary vascular resistance. Elevated LA or ventricular end-diastolic pressures do not change the transpulmonary gradient. A low fenestration gradient is associated with hypovolemia.

37. (C) The murmur described in the examination is continuous and it peaks around the second heart sound—typical of a PDA. An audible PDA usually can be visualized with transthoracic echocardiography. If that imaging is suboptimal, CT or MRI of the chest may be helpful. If the PDA is audible, especially in a patient with a wide pulse pressure, one would expect left ventricular and left atrial enlargement. This patient should undergo closure of the PDA, usually via a transcatheter procedure.

38. (D) The continuous murmur is typical of a PDA or systemic arterial to pulmonary artery collateral vessels (common in patients with pulmonary atresia). Patients with PA/VSD frequently have a right-sided aortic arch and the PDA may be best heard over the right back. A right coronary fistula may produce a continuous murmur but is unlikely to be heard best in the back. The other choices do not generate continuous murmurs.

39. (E) The patient has an ASD that technically would be amenable to device closure. But, other issues need to be addressed such as intervention for the tricuspid valve—dictating that the patient is best served with surgical management. Patients who may need additional cardiac surgery for coronary revascularization, valve repair, or arrhythmia surgery should be treated with surgery rather than sole transcatheter device closure of the ASD.

40. (E) The patient has two sources of pulmonary blood flow, the SVC exclusively supplies the RPA, the IVC flow via the Fontan conduit supplies the LPA exclusively. In addition, the patient has substrate for right lung fistula due to the classic Glenn procedure. Pulmonary blood flow cannot be accurately quantitated nor can pulmonary vascular resistance be assessed.

41. (C) Polymerase chain reaction is needed to confirm presence of active infection. Antibody positivity indicates immunization or prior infection. Prior to 1992, no formal testing was available for what was formerly called “non A-non B hepatitis.” It is estimated that 5% of patients with congenital heart disease who had cardiac surgery prior to 1992 are infected with hepatitis C.

42. (A) Serum BNP levels >140 pg/mL correlated with poor long-term outcome in patients with Eisenmenger syndrome (Reardon LC, et al. Am J Cardiol 2012; 110:1523-6). Elevated BNP correlates with ventricular dilation, dysfunction, and heart failure symptoms but has not been studied specifically in the other lesions listed. Although based on data from just one study, this question has appeared on several board examinations.

43. (A) The patient in this scenario has a primum ASD. A primum ASD is located in the most anterior and inferior aspect of the atrial septum at the level of the mitral and tricuspid valves. It is associated with a cleft mitral valve. Any ASD with moderate right ventricular volume overload should be closed. There is decreased mortality after surgical closure (compared to medical treatment), although nonfatal cardiovascular complications are similar. Unlike secundum ASDs, primum ASDs should not be closed using a device in the cath lab. Although there is right atrial enlargement, the patient is not hypertensive and there is no definitive role for ACE inhibition in shunt lesions. Patients with primum ASDs will frequently have left axis deviation on ECG that is related to the lesion rather than any coronary artery disease. There is no indication for a cardiac cath (unless there is concern for pulmonary hypertension).

44. (E) Any patient who survived a cardiac arrest due to nonreversible causes should have an ICD placed. There is no evidence of a reversible cause in this patient. Ablation may be offered as an alternative in a patient with a slow, stable, monomorphic ventricular tachycardia, but not the fast ventricular tachycardia resulting in cardiac arrest seen in this patient. The incidence of ICD complications was reported to be 30% in one study of patients with tetralogy of Fallot, compared to about 10% in the postmyocardial infarction population. The incidence of inappropriate shocks in tetralogy of Fallot is about 25%, which is similar to that seen in other congenital heart lesions. Antiarrhythmic medications are not as effective as an ICD in preventing recurrent arrhythmias. Beta blockers, amiodarone, and sotalol do not decrease the risk of appropriate ICD shocks in patients with tetralogy of Fallot. A transvenous system can be performed in a patient with free pulmonary regurgitation. Patients with residual intracardiac shunts should not have transvenous pacing/ICD leads.

45. (D) TBX5 mutation is associated with Holt-Oram syndrome. It is inherited in an autosomal dominant fashion. TGFBR 1 & 2 with Loeys-Dietz; FBN1 with Marfan syndrome; Trisomy 21 is Down syndrome; NKX 2.5 with ASD + heart block.

46. (A) NKX 2.5 mutation is associated with familial occurrence of ASDs and progressive AV block. The GATA IV mutation is associated with ASDs without AV block. TBX5 is associated with Holt-Oram syndrome, NOTCH mutations are associated with AVSD.

47. (A) The embryologic origin of the valve of the fossa ovalis is derived from septum primum. The superior limbus and inferior limbus originate from the septum secundum. Atrial appendage morphology is not related to development of the atrial septum. The endocardial cushions are important in septation of the atrioventricular septum and delamination of the atrioventricular valves.

48. (C) The cardiac disease in pregnancy (CARPREG) investigators demonstrated in a prospective multicenter study that maternal cardiac risk could be predicted with the use of a risk index. Cardiac events were defined as pulmonary edema, arrhythmia, stroke, or cardiac death. The four predictors of primary cardiac events were (1) prior cardiac event, (2) baseline NYHA class >II or cyanosis, (3) left heart obstruction (mitral valve area <2 cm², aortic valve area <1.5 cm², or peak LVOT gradient >30 mm Hg by echocardiography), and (4) reduced systemic ventricular systolic function (EF <40%). The risk of maternal cardiac complication with zero predictors was 5%, with one predictor it was 25%, and with greater than one predictor the risk was 75%. The risk score was further refined by the Boston Adult Congenital Heart Disease Group in a study that demonstrated that including decreased subpulmonary ventricular function and/or severe pulmonary regurgitation as a predictor in the risk index improved the accuracy of the assessment. Of the answers listed, the patient described in (C) has the highest predicted risk with a prior cardiac event (history of atrial fibrillation) and severe pulmonary valve regurgitation. Her poor peak VO₂ may be a further indication of poor outcome, even if she has no complaints clinically. The 32-year-old patient has no predictors. The 30-year-old patient has one predictor. The 20-yearold patient could be considered to have one predictor—poor subpulmonary ventricular function.

49. (C) ACE inhibitors can cause fetal renal dysfunction in the third trimester, but have also been demonstrated to be a teratogen. Therefore, ACE inhibitors should be avoided throughout pregnancy. ARBs should be considered to have the same risk profile and should be avoided. Hydralazine and nitrates are safe in pregnancy and together provide similar physiologic response to ACE inhibition.