A 34-year-old man comes to the office due to frequent headaches. A year ago, he was diagnosed with migraines and prescribed painkillers without significant relief. Recently, the patient has had high blood pressure on several checks, but he has no other significant medical history. He does not use tobacco, alcohol, or recreational drugs. The patient has no family history of hypertension or cardiovascular disease. Blood pressure is 185/105 mm Hg and pulse is 88/min and regular. A fourth heart sound is heard on cardiac auscultation. A continuous murmur is noted throughout the thorax in multiple areas. The lungs are clear. Which of the following is most likely to be found on chest x-ray?
Coarctation of the aorta | |
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This patient most likely has coarctation of the aorta, a usually congenital narrowing of the descending aorta typically located just distal to the left subclavian artery.
The narrowing creates a proximal arterial pressure load affecting the upper body. Patients can sometimes present in adulthood, typically with asymptomatic hypertension (of the upper extremities) and less often with accompanying epistaxis and headaches. There may be a systolic murmur (due to blood flow through a constricted aorta) heard at the left infraclavicular area anteriorly and left interscapular area posteriorly (sometimes difficult to auscultate in the supine position); the murmur may be continuous if collateral vessels are present. A fourth heart sound (due to hypertension-induced left ventricular hypertrophy) may also be present. Brachial-femoral pulse delay, if measured, is typically present with diminished femoral pulses.
In the majority of patients, chest x-ray reveals notching of the posterior third of the third to eighth ribs that represents bony erosion due to enlarged intercostal arteries. A classic "3" sign created by indentation of the aorta with pre- and poststenotic dilation may also be present.
(Choice A) Turner syndrome carries an association with kyphoscoliosis, which is characterized by curvature of the spine in 2 planes (anteroposterior and lateral). Approximately 30% of patients with Turner syndrome will also have coarctation of the aorta. However, Turner syndrome is only seen in women.
(Choice B) Diffuse vascular calcifications are seen in patients with premature atherosclerosis and in those with advanced renal disease. There is no such history in this patient.
(Choice D) Prominent right atrial contour is often seen in Ebstein congenital abnormality (apical displacement of the tricuspid valve with atrialization of the right ventricle). Patients with long-standing coarctation of the aorta may develop left-sided cardiac enlargement, but right-sided cardiac enlargement is not typical.
(Choice E) Upturning of the cardiac apex ("boot-shaped heart") occurs in tetralogy of Fallot due to pulmonic stenosis and consequent right ventricular hypertrophy. Patients typically present with cyanosis early in life.
Educational objective:
Coarctation of the aorta is a narrowing of the descending aorta that leads to a proximal arterial pressure load. Patients typically present with upper extremity hypertension and diminished femoral pulses with brachial-femoral delay. Chest x-ray usually demonstrates inferior notching of the third to eighth ribs due to pressure-induced enlargement of the intercostal arteries.