A newborn is diagnosed with tetralogy of Fallot. The mother reveals she took high doses of isotretinoin, a retinoid-like drug, during her first trimester of pregnancy. The teratogenic effect of this medication most likely interfered with which critical cell population during cardiac development?
A 16-year-old with end-stage heart failure secondary to a palliated single ventricle is being evaluated for cardiac transplantation. As part of the pre-transplant workup, he undergoes a cardiac catheterization to assess his hemodynamic status. Which of the following findings would be a significant contraindication for an isolated heart transplant and would lead to consideration for a heart-lung transplant?
An 8-month-old infant with a large, un-repaired ventricular septal defect presents with tachypnea, poor feeding, and diaphoresis. You diagnose heart failure due to volume overload. What is the most appropriate first-line pharmacologic therapy to manage this patient’s congestive symptoms?
A 10-year-old boy with difficulty climbing stairs is being evaluated. A cardiac screen is performed due to his suspected neuromuscular disorder. An echocardiogram shows a dilated left ventricle with moderately reduced systolic function. Which condition is a common cause of dilated cardiomyopathy in boys?
A 3-year-old girl with a history of a Fontan procedure for tricuspid atresia presents with a 2-month history of peripheral edema, ascites, and diarrhea. Laboratory evaluation reveals hypoalbuminemia. Which of the following is the most likely late complication of her single-ventricle palliation causing this presentation?
A 2-week-old neonate presents with hydrops fetalis, severe respiratory distress, and refractory heart failure. A radiograph reveals diffuse calcification of the major arteries. The clinical picture is highly suspicious for generalized arterial calcification of infancy. Pathogenic variants in which gene account for the majority of cases of this rare and often lethal disorder?
An obese 14-year-old is diagnosed with stage 1 primary hypertension without evidence of target organ damage during pre-participation sports physical. What is the cornerstone of initial therapy for this adolescent?
A 3-month-old infant is brought to the emergency department with a 24-hour history of poor feeding, irritability, and an ashen color. The heart rate is sustained at 280 beats/min, and he has low blood pressure, hepatomegaly and poor peripheral pulses. An ECG confirms supraventricular tachycardia. Given the signs of severe heart failure, what is the most appropriate initial management?
A 2-month-old infant is brought to the emergency department with episodes of paroxysmal irritability, pallor, and diaphoresis, particularly after feeding. An electrocardiogram (ECG) shows a QR pattern in leads I and aVL with inverted T waves. Echocardiography reveals a dilated left ventricle with poor function. What is the most likely diagnosis?
A 17-year-old is 10 years post-heart transplant. An annual surveillance coronary angiogram reveals severe, diffuse narrowing of the distal coronary arteries, consistent with cardiac allograft vasculopathy (CAV). The patient has no symptoms of angina. Which statement most accurately describes the pathophysiology and management of this condition?
