Fibromuscular dysplasia of the coronary arteries: a rare cause of death in infants and young children

Abstract

Fibromuscular dysplasia is a common vascular disease, which mainly affects the renal arteries. In this study, we report the case of a 1-day-old infant who presented with severe left ventricular dysfunction immediately after birth, and subsequently died 3 days postnatally. At autopsy, an extensive myocardial infarction of the left ventricle and the septum was found, caused by an isolated fibromuscular dysplasia of left coronary artery.

2 Cases of Spontaneous Coronary Artery Dissection in Neonates

“…The first was a 1-day-old with LV dysfunction who was found to have concentric collagen accumulation in the media and adventitial layers of the LAD. 5 The second was a 5-month-old in whom both coronary arteries were cord-like and showed luminal narrowing with diffuse thickening through the smooth muscle layer. 6 A 2-day-old infant with tetralogy of Fallot was found to have coronary artery FMD, with the classic “string of beads” appearance, but without histologic confirmation.…”

Fibromuscular Dysplasia of the Coronary Arteries: An Infrequent Cause of Sudden Death

“…In childhood, there are few reported cases. Two of the cases described occurred in newborns, manifesting as cardiogenic shock at birth, both died and the diagnosis was made at autopsy [3,4]. The other two cases occurred in two 12-year-old adolescents, one of them presented SCD, diagnosed after autopsy [5] and the other presented AMI, surviving after performing percutaneous transluminal coronary angioplasty [6].…”