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Chest Pain in a 39-Year-Old Man: What Could Be the Underlying Cause?

(A) Computed tomography coronary angiography showing the characteristic multifocal “string of beads” appearance with beading (arrows) that represents areas of stenosis alternating with dilatation. (B) Coronary angiography image showing the exact location (arrows) where the drug-eluting stents were placed. LAD = left anterior descending artery; LCx = left circumflex artery; RCA = right coronary artery.

(A) Computed tomography coronary angiography showing the characteristic multifocal “string of beads” appearance with beading (arrows) that represents areas of stenosis alternating with dilatation. (B) Coronary angiography image showing the exact location (arrows) where the drug-eluting stents were placed. LAD = left anterior descending artery; LCx = left circumflex artery; RCA = right coronary artery.

 

A 39-year-old man with unremarkable past medical history, but with a positive family history for coronary artery disease, was admitted to the hospital because of persistent arterial hypertension and sudden-onset chest pain in the mid-substernal region with neck radiation.

Assessment

Results from physical examination were unremarkable. The 12-lead electrocardiogram and the cardiac biomarkers were within normal limits. Computed tomography coronary angiography revealed a multifocal “string of beads” appearance of the coronary arteries (Figure A). Angiographic computed tomography of the aorta revealed only insignificant proximal stenosis of an accessory renal artery.

Diagnosis

A diagnosis of isolated coronary artery fibromuscular dysplasia was undertaken, and 2 drug-eluting stents were placed in the left descending and left circumflex arteries (Figure B).

Management

Fibromuscular dysplasia with coronary artery involvement should be considered in young individuals without cardiac or genetic risk factors who present with acute coronary syndrome and/or persistent arterial hypertension.1,2 The diagnosis of fibromuscular dysplasia of the coronary arteries is challenging. Several conditions need to be carefully considered and excluded before the definite diagnosis of fibromuscular dysplasia is made. The differential diagnosis should include polyarteritis nodosa, Ehlers-Danlos syndrome type VI, Takayasu’s arteritis, coronary vasospasm, myocardial bridging, and atherosclerotic plaque.2 In the majority of patients, a catheter-based angiography in combination with intravascular ultrasound is a fundamental part of evaluating and treating cardiac ischemia. Early and accurate diagnosis of fibromuscular dysplasia is important, especially when stenting of the affected vascular beds is necessary for preventing potentially lethal consequences.

 

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-Sotiris C. Plastiras, MD, PhD, Evangelia Zampeli, MD, PhD, Haralampos M. Moutsopoulos, MD, Master ACR

This article originally appeared in the December 2016 issue of The American Journal of Medicine.

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