According to ACC/AHA guidelines for the management of adults with congenital heart disease (ACHD), what is the primary hemodynamic threshold that mandates closure of an isolated Atrial Septal Defect (ASD) secondum type in the absence of severe irreversible pulmonary hypertension?
Q2
A 32-year-old female with an unrepaired secundum ASD presents with progressive dyspnea and cyanosis. Cardiac catheterization reveals pulmonary arterial pressure of 95 mmHg and pulmonary vascular resistance (PVR) of 11 Wood units, with net right-to-left shunting. What is this clinical syndrome called, and is device closure advised?
Q3
Which anatomical type of Atrial Septal Defect is most commonly associated with a cleft mitral valve and a significant risk of early-onset mitral regurgitation?
Q4
Which congenital cardiac anomaly is classically associated with Scimitar syndrome (hypogenetic lung syndrome)?
Q5
A patient presents with a continuous ‘machinery’ murmur best heard at the left upper sternal border, bounding peripheral pulses, and a wide pulse pressure. What is the standard guideline recommendation for an isolated Patent Ductus Arteriosus (PDA) in an adult with left heart volume overload?
Q6
Tetralogy of Fallot (ToF) is characterized by four hallmark anatomical features. Which of the following correctly lists all four primary components?
Q7
Long-term follow-up of surgically repaired Tetralogy of Fallot patients in adulthood frequently reveals a specific chronic hemodynamic complication resulting from prior transannular patch repair or infundibulotomy. What is this primary lesion?
Q8
Transposition of the Great Arteries (TGA) with concordant atrioventricular and discordant ventriculoarterial connections (D-TGA) was historically repaired using an atrial baffle operation (Mustard or Senning procedure). What is the major long-term structural complication unique to patients who underwent an atrial baffle repair?
Q9
Congenitally Corrected Transposition of the Great Arteries (L-TGA or ccTGA) involves both atrioventricular and ventriculoarterial discordance. What is the defining long-term natural history risk for patients with unoperated ccTGA?
Q10
Ebstein Anomaly is characterized by failure of delamination of the tricuspid valve leaflets, leading to downward displacement of the septal and posterior leaflets into the right ventricle. Which electrocardiographic finding is classically associated with Ebstein anomaly and predisposes patients to paroxysmal supraventricular tachycardias?
Q11
Coarctation of the aorta is classically localized near the insertion of the ligamentum arteriosum. Which clinical sign is characteristic of significant aortic coarctation on physical examination?
Q12
A 24-year-old male with Turner syndrome is evaluated for cardiovascular abnormalities. Beyond bicuspid aortic valve, what is the most critical life-threatening cardiovascular aortopathy associated with Turner syndrome?
Q13
Marfan syndrome is an autosomal dominant connective tissue disorder caused by mutations in the FBN1 gene (fibrillin-1). What is the primary cardiovascular indication for elective surgical replacement of the aortic root in Marfan syndrome?
Q14
Patients with Cyanotic Congenital Heart Disease (e.g., unrepaired Tetralogy of Fallot or Eisenmenger syndrome) frequently develop secondary erythrocytosis (polycythemia) as a physiological adaptation to chronic hypoxemia. What is the fundamental guideline recommendation regarding routine therapeutic phlebotomy in these patients?
Q15
Pregnancy in women with congenital heart disease carries varying degrees of maternal risk, classified using the World Health Organization (WHO) risk stratification framework. Which of the following conditions is classified as WHO Risk IV (Pregnancy Contraindicated)?
Q16
A 28-year-old male with a history of surgically repaired coarctation of the aorta in childhood presents for routine ACHD evaluation. His office blood pressure is 155/90 mmHg in the right arm. What is the recommended initial diagnostic test to evaluate for recurrent (residual) coarctation or pseudoaneurysm formation at the repair site?
Q17
Fontan circulation is a palliative surgical procedure utilized for patients with single ventricle physiology (e.g., hypoplastic left heart syndrome, tricuspid atresia). What is the fundamental hemodynamic characteristic of a Fontan circulation?
Q18
A patient with a Fontan circulation presents with chronic peripheral edema, severe hypoalbuminemia, ascites, and chronic diarrhea. What is this classic protein-losing enteropathy (PLE) syndrome primarily driven by?
Q19
Sinus Valsalva Aneurysms are congenital or acquired dilations of the aortic sinuses. When a Sinus of Valsalva aneurysm ruptures, into which cardiac chamber or space does it most commonly rupture, producing a continuous murmur and acute heart failure?
Q20
Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA / Bland-Garland-White syndrome) typically presents in infancy when pulmonary vascular resistance drops. However, some patients present in adulthood. What collateral circulation pattern allows these adult survivors to reach adulthood?
Q21
Persistent Left Superior Vena Cava (PLSVC) is the most common congenital thoracic venous anomaly. Into which cardiac structure does an isolated PLSVC typically drain?
Q22
According to ACC/AHA ACHD guidelines, infective endocarditis (IE) prophylaxis is recommended prior to dental procedures for which specific high-risk category of adult congenital heart disease patients?
Q23
A 22-year-old female with a history of surgically repaired Tetralogy of Fallot presents with palpitations and sudden-onset sustained monomorphic ventricular tachycardia (VT). What is the most common electrophysiological mechanism underlying VT in repaired ToF?
Q24
Which congenital heart defect is classically associated with DiGeorge syndrome (22q11.2 microdeletion)?
Q25
A 30-year-old male with a history of a small perimembranous VSD presents with persistent fever, weight loss, and new-onset aortic regurgitation. Blood cultures grow Streptococcus viridans. What is the classic echocardiographic finding and mechanism of aortic valve involvement in perimembranous VSD complicated by infective endocarditis?
