Review Question - QID 214498

This patient is presenting with hypertension refractory to medications, atrophy of her lower extremities, and a chest radiograph demonstrating inferior rib notching, which is concerning for coarctation of the aorta. Coarctation of the aorta is associated with a bicuspid aortic valve.

Coarctation of the aorta occurs when there is a narrowing of the aorta that leads to the classic presentation of hypertension in the upper extremities and hypotension and atrophy of the lower extremities. Blood is shunted through collateral vessels including the intercostal arteries that can lead to inferior rib notching. The diagnosis can be supported with a transesophageal echocardiogram or a CT angiogram of the chest, and treatment involves surgical correction of the coarctation. Coarctation of the aorta can be associated with other medical conditions such as a bicuspid aortic valve, Turner syndrome (45,XO), and a ventricular septal defect.

Figure/Illustration A demonstrates a chest radiograph with notching inferior to the ribs (red arrows) which is concerning for aortic coarctation. Increased pressure in the intercostal arteries leads to this finding.

Incorrect Answers:
Answer 1: Adrenal malignancy describes a pheochromocytoma that would present with episodic headaches, palpitations, anxiety, and hypertension secondary to catecholamine release. Workup includes urine/plasma metanephrines, a CT scan to localize the tumor, and treatment includes phenoxybenzamine and surgical removal of the mass.

Answer 3: Connective tissue disease (e.g., Marfan syndrome) could predispose patients to mitral valve proplase (a midsystolic click heard best at the apex) or aortic regurgitation (a diastolic decrescendo murmur best heard at the right upper sternal border). Other findings in aortic regurgitation include head bobbing, water hammer pulses, fingernail pulsations, and a widened pulse pressure.

Answer 4: Impaired renal blood flow could be seen in fibromuscular dysplasia causing renal artery stenosis, which would lead to pathologic activation of the renin-angiotensin-aldosterone system, a metabolic alkalosis, and hypokalemia. This could explain this patient’s hypertension; however, it would not explain her rib notching or lower extremity atrophy.

Answer 5: XXY chromosome could be seen in Klinefelter syndrome which would present with a tall male patient with gynecomastia and testicular atrophy. On the other hand, Turner syndrome (45,XO) patients would be at increased risk for aortic coarctation.

Bullet Summary:
Coarctation of the aorta is associated with a bicuspid aortic valve.