ABIM Adult Congenital Heart Disease

In the staged palliation of hypoplastic left heart syndrome (HLHS), which procedure constitutes Stage 1 (neonatal)?

• ABidirectional Glenn anastomosis
• BNorwood procedure with Sano or BT shunt
• CTotal cavopulmonary Fontan completion
• DArterial switch operation

Which of the following best describes Fontan circulation hemodynamics?

• APulmonary blood flow is driven by a subpulmonic ventricle
• BPulmonary blood flow depends on passive flow from systemic veins with obligate elevation…
• CSystemic venous return bypasses the lungs entirely
• DPulmonary artery pressure is typically subnormal (<5 mmHg)

A 28-year-old man with a fenestrated lateral tunnel Fontan presents with peripheral edema, chronic diarrhea, and serum albumin 2.0 g/dL. Which diagnostic test is most specific for protein-losing enteropathy (PLE)?

• ASerum total protein
• B24-hour urine protein
• CFecal alpha-1 antitrypsin clearance
• DStool osmotic gap

Which of the following is a recognized late complication of the Fontan circulation?

• APulmonary stenosis
• BFontan-associated liver disease (FALD) with cirrhosis and hepatocellular carcinoma risk
• CBronchiectasis
• DSevere aortic regurgitation

A 35-year-old with a Fontan palliation presents with expectoration of rubbery bronchial casts. What is this complication called?

• AAllergic bronchopulmonary aspergillosis
• BPlastic bronchitis
• CDiffuse alveolar hemorrhage
• DCryptogenic organizing pneumonia

Which of the following is NOT a classic component of tetralogy of Fallot?

• APulmonary stenosis (RVOT obstruction)
• BOverriding aorta
• CVentricular septal defect
• DAtrial septal defect

In an adult with repaired tetralogy of Fallot (transannular patch repair in infancy), what is the most common hemodynamic lesion contributing to late RV dilation?

• AResidual VSD
• BPulmonary stenosis
• CSevere pulmonary regurgitation
• DTricuspid stenosis

According to current ACHD guidelines, which of the following supports pulmonary valve replacement (PVR) in an asymptomatic adult with repaired TOF and severe PR?

• ARVEDVi >=160 mL/m2 or RVESVi >=80 mL/m2
• BLVEF 55%
• CRV/LV volume ratio 1.2
• DMild RV dilation without other findings

A 32-year-old with d-transposition of the great arteries repaired with a Mustard procedure as a child now has progressive dyspnea and an LVEF of 30% measured on MRI. Which ventricle is this 'LVEF'?

• AThe morphologic left ventricle, which is the systemic pumping chamber
• BThe morphologic right ventricle, which is the systemic pumping chamber
• CThe common ventricle in a single-ventricle heart
• DThe subpulmonic left ventricle

Which of the following distinguishes the arterial switch (Jatene) procedure from the atrial switch (Mustard/Senning) for d-TGA?

• AThe arterial switch restores the morphologic LV as the systemic ventricle
• BThe arterial switch creates atrial baffles
• CThe arterial switch is only used in adults
• DThe arterial switch requires a Fontan completion

In congenitally corrected transposition of the great arteries (l-TGA / ccTGA), which statement is true?

• AThe morphologic right ventricle pumps to the aorta
• BThe morphologic left ventricle pumps to the aorta
• CThere is always a concordant atrioventricular connection
• DSystemic venous return drains to the aorta

A 42-year-old man had coarctation repair at age 5. He now has resistant hypertension. Which is the most important surveillance finding to assess?

• ALeft atrial size
• BRecoarctation gradient and ascending aortic aneurysm/dissection risk
• CTricuspid regurgitation severity
• DMitral annular calcification

Which ASD subtype is most common?

• AOstium secundum
• BOstium primum (AVSD type)
• CSinus venosus
• DCoronary sinus

A sinus venosus ASD is most commonly associated with which additional anomaly?

• APatent ductus arteriosus
• BAnomalous pulmonary venous return (usually right upper lobe to SVC)
• CCoarctation of the aorta
• DCoronary ostial stenosis

What Qp:Qs ratio is generally used as a threshold supporting closure of a hemodynamically significant atrial septal defect in an adult?

• A>=1.0
• B>=1.2
• C>=1.5
• D>=3.0

Which type of VSD is most common?

• APerimembranous
• BMuscular
• CInlet (AV canal type)
• DOutlet (supracristal/subarterial)

An outlet (supracristal / subarterial / doubly committed) VSD is most strongly associated with which complication?

• ATricuspid regurgitation
• BAortic cusp prolapse with progressive aortic regurgitation
• CMitral stenosis
• DCoronary sinus dilation

A complete atrioventricular septal defect (AVSD) is most commonly associated with which genetic syndrome?

• ATurner syndrome
• BDiGeorge (22q11.2 deletion)
• CDown syndrome (trisomy 21)
• DWilliams syndrome

Eisenmenger syndrome is best defined as:

• AReversal of a left-to-right shunt due to severe pulmonary vascular disease (PAH) causing…
• BCongenital absence of the pulmonary valve
• CA form of restrictive cardiomyopathy
• DAn anomalous origin of the left coronary from the pulmonary artery

Which PAH-targeted therapy class is generally first-line for Eisenmenger syndrome based on the BREATHE-5 trial?

• ACalcium channel blockers (nifedipine)
• BEndothelin receptor antagonist (bosentan)
• CIntravenous epoprostenol only
• DLoop diuretics

In Eisenmenger syndrome, why is aggressive IV fluid resuscitation or sudden afterload reduction dangerous?

• AIt increases the right-to-left shunt and worsens cyanosis
• BIt decreases right-to-left shunt and causes pulmonary edema
• CIt has no hemodynamic effect
• DIt improves saturation but causes hypertension

A 27-year-old woman with Eisenmenger syndrome asks about pregnancy. What is the expected maternal mortality?

• A<1%
• B5-10%
• C30-50%
• DPregnancy is not higher risk than in the general population

The patent foramen ovale (PFO) is present in approximately what percentage of the general adult population?

• A1%
• B5%
• C25%
• D75%

Which landmark trial supported PFO closure over medical therapy for secondary prevention of cryptogenic stroke in patients <60 years?

• ARESPECT (and subsequent trials CLOSE and REDUCE)
• BPARTNER
• CSPRINT
• DJUPITER

Ebstein's anomaly is characterized by:

• AApical displacement of the septal and posterior tricuspid leaflets creating an 'atrialize…
• BAbsence of the pulmonary valve
• CCleft anterior mitral valve leaflet
• DAnomalous origin of the left coronary from the pulmonary artery

A 24-year-old woman with Ebstein's anomaly presents with palpitations and a delta wave on ECG. The most likely arrhythmia substrate is:

• AAV nodal re-entrant tachycardia
• BWolff-Parkinson-White / accessory pathway (often multiple, right-sided)
• CAtrial flutter with 2:1 block
• DSinus tachycardia with aberrancy

Hypertrophic cardiomyopathy (HCM) is most commonly caused by mutations in which sarcomere protein gene?

• AMYH7 (beta-myosin heavy chain) or MYBPC3 (myosin-binding protein C)
• BLMNA (lamin A/C)
• CDMD (dystrophin)
• DFBN1 (fibrillin-1)

A 19-year-old competitive basketball player has HCM with an LVOT gradient of 60 mmHg at rest and NYHA III symptoms despite metoprolol and disopyramide. What is the next step?

• ACardiac transplantation
• BSeptal reduction therapy (surgical myectomy or alcohol septal ablation) at an expert cent…
• CDual chamber pacing
• DObservation with beta-blocker titration

Which of the following is a major risk factor for sudden cardiac death in HCM that supports primary prevention ICD?

• ASeptal thickness 10 mm
• BPrior cardiac arrest or sustained VT
• CNormal exercise BP response
• DLVEF 70%

Left ventricular noncompaction (LVNC) is characterized by:

• AProminent LV trabeculations with deep intertrabecular recesses communicating with the LV…
• BConcentric LV hypertrophy without trabeculations
• CA dilated right ventricle with fibrofatty replacement
• DA restrictive cardiomyopathy pattern

A bicuspid aortic valve is associated with which aortopathy pattern?

• ADilation of the descending thoracic aorta only
• BDilation of the aortic root and ascending aorta, independent of valve hemodynamics
• CAbdominal aortic aneurysm
• DDissection of the left subclavian artery

Marfan syndrome is caused by mutations in which gene?

• AFBN1 (fibrillin-1)
• BTGFBR2
• CCOL3A1
• DACTA2

In a patient with Marfan syndrome and no family history of early dissection, at what aortic root diameter is elective aortic root replacement generally recommended?

• A3.5 cm
• B4.0 cm
• C5.0 cm
• D7.0 cm

Which medications are considered first-line for slowing aortic root growth in Marfan syndrome?

• ABeta-blockers (and/or losartan — an angiotensin receptor blocker)
• BACE inhibitors alone
• CCalcium channel blockers (dihydropyridines) at high dose
• DThiazide diuretics

Loeys-Dietz syndrome is caused by mutations in which genes?

• AFBN1
• BTGFBR1 or TGFBR2 (also SMAD3, TGFB2, TGFB3)
• CCOL3A1
• DACTA2 only

In the WHO classification of pulmonary hypertension, which group includes PAH due to congenital heart disease?

• AGroup 1 (PAH) — specifically 1.4 (PAH associated with CHD)
• BGroup 2 (left heart disease)
• CGroup 3 (lung disease/hypoxia)
• DGroup 4 (CTEPH)

The mWHO (modified World Health Organization) pregnancy risk classification places which of the following in class IV (pregnancy contraindicated)?

• ARepaired small ASD
• BMild pulmonic stenosis
• CSevere pulmonary arterial hypertension / Eisenmenger syndrome
• DBicuspid aortic valve with root <4.0 cm

A 30-year-old cyanotic ACHD patient has hemoglobin 22 g/dL, hematocrit 65%, and symptoms of headache and visual disturbances (hyperviscosity). Appropriate management includes:

• AAggressive phlebotomy to a target hematocrit of 45%
• BCautious isovolumic phlebotomy with simultaneous volume replacement if symptoms are sever…
• CIron supplementation regardless of iron studies
• DImmediate surgical shunt closure

A patient with Eisenmenger physiology is undergoing elective noncardiac surgery. Which intraoperative principle is most important?

• AMaintain systemic vascular resistance; avoid sudden afterload drop and air in IV lines
• BAggressive fluid loading to >30 mL/kg
• CInduce deliberate hypotension to reduce shunt
• DUse nitrous oxide to lower PVR

Pulmonary artery pressure was measured on right heart catheterization. Which of the following defines precapillary pulmonary hypertension under current definitions?

• AMean PAP >20 mmHg, PAWP <=15 mmHg, PVR >2 Wood units
• BMean PAP >40 mmHg only
• CSystolic PAP >50 mmHg only
• DPAWP >15 mmHg and PVR <2 Wood units

Transcatheter pulmonary valve replacement with the Melody or Harmony valve is most commonly performed for:

• ANative pulmonary stenosis in infants
• BDysfunctional RV-to-PA conduits or severe PR in repaired conotruncal lesions (e.g., TOF,…
• CPulmonary valve endocarditis as first-line therapy
• DTricuspid regurgitation

A patent ductus arteriosus produces what murmur on auscultation?

• AContinuous 'machinery-like' murmur at the left infraclavicular area
• BHolosystolic murmur at the apex radiating to the axilla
• CMid-diastolic rumble at the apex
• DEjection systolic murmur at the right upper sternal border

In total anomalous pulmonary venous return (TAPVR), which is the most common drainage pattern?

• ASupracardiac (drainage to the innominate vein via a vertical vein to the SVC)
• BInfracardiac (drainage to the portal system)
• CCardiac (to the coronary sinus)
• DMixed

A 29-year-old with repaired TOF is asymptomatic. MRI shows RVEDVi 180 mL/m2, RVESVi 90 mL/m2, RVEF 42%, LVEF 55%, severe PR. What is the appropriate next step?

• AObserve and repeat MRI in 3 years
• BRefer for pulmonary valve replacement (surgical or transcatheter) at an ACHD center
• CStart beta-blocker therapy only
• DPerform cardiac transplantation evaluation

A 40-year-old with prior Mustard repair for d-TGA presents with new atrial flutter. What is the most appropriate management?

• ARate control only; no need for anticoagulation
• BAnticoagulation and referral for atrial flutter ablation at an ACHD electrophysiology cen…
• CEmpiric amiodarone indefinitely
• DObservation

Which of the following is the strongest indication for infective endocarditis prophylaxis in ACHD?

• ARepaired secundum ASD with no residual defect, 10 years after device closure
• BBicuspid aortic valve with mild AS and no prior endocarditis
• CProsthetic cardiac valve, prior infective endocarditis, cyanotic unrepaired CHD, or first…
• DSmall PFO with no stroke

A 26-year-old woman has had her first ACHD pregnancy counseling visit. She has a repaired coarctation with residual mild gradient and a bicuspid aortic valve with ascending aorta 3.8 cm and no AR/AS. What is her mWHO pregnancy class?

• AmWHO I (no or minimal increased risk)
• BmWHO II (small increase in maternal morbidity/mortality)
• CmWHO III (significantly increased risk)
• DmWHO IV (pregnancy contraindicated)