Congenital Heart Disease USMLE Step 1 Practice Question
A 4-year-old boy with a family history of sudden cardiac death presents to the emergency department with acute onset dyspnea and chest discomfort after playing soccer. His mother reports he has always been unusually tall for his age. Vital signs: BP 88/32 mmHg, HR 125/min, RR 32/min, SpO2 97% on room air. Physical examination reveals a hyperdynamic precordium, a wide pulse pressure, and an early diastolic decrescendo murmur best heard at the left sternal border. Chest X-ray shows marked cardiomegaly with pulmonary edema. Transthoracic echocardiography demonstrates an ascending aorta measuring 3.2 cm (z-score >3) with severe aortic regurgitation. Which of the following best explains his presentation?
Answer choices
- AAortic regurgitation secondary to aortic root dilation and annuloaortic ectasiaCorrect answer
- BAcute aortic dissection with secondary aortic regurgitation
- CBicuspid aortic valve with progressive aortic stenosis
- DPatent ductus arteriosus with left-to-right shunting
- EMitral valve prolapse with secondary left ventricular dysfunction
- FCoarctation of the aorta with acute decompensation
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