2 Cases of Spontaneous Coronary Artery Dissection in Neonates

Abstract

Spontaneous coronary artery dissection in infants is a rare phenomenon. We present 2 neonates with severe ventricular dysfunction due to coronary artery dissection. Neither patient had evidence of extracardiac fibromuscular dysplasia or other comorbidities that would explain the presentation. (Level of Difficulty: Advanced.).

Keywords: ACTN2, alpha-actinin 2; ECMO, extracorporeal membrane oxygenation; FMD, fibromuscular dysplasia; LAD, left anterior descending artery; LCA, left coronary artery; LV, left ventricular; MCA, middle cerebral artery; PCA, posterior cerebral artery; RCA, right coronary artery; RV, right ventricular; SCAD, spontaneous coronary artery dissection; SVT, supraventricular tachycardia; coronary artery; heart failure; spontaneous coronary artery dissection.

Graphical abstract

Figure 1

Left Anterior Descending Artery (LAD) Pathology (A) Macroscopic sections of proximal to mid-LAD (left to right). Left zoomed section demonstrates a focal white fibrous solid area without visible vascular lumen (black arrow) and a faint brown area of adjacent hemorrhage. Right zoomed section demonstrates perivascular hemorrhage, represented by a dark brown area underneath the slit-like coronary artery within the epicardial fat (red arrow). (B) LAD shows 3 distinct layers: adventitia (A), media (M), and intima (I). Intimal hyperplasia with disruption of internal elastic lamina (black arrow points to intact elastic lamina; if traced circumferentially, the disrupted region is between the 2 triangles) and tunica media, and prominent adventitial and medial fibrosis (asterisk) at the site of injury/dissection, with resultant narrowing of the lumen, are shown. Perivascular hemorrhage (star) corresponds to area of hemorrhage in A, as the patient was taking aspirin and bivalirudin at time of transplantation. Between the adventitial and medial layers, a plane of separation (dashed line) is seen focally with a much larger gap and disruption toward the right side. (C) Close-up view of disrupted internal elastic lamina (arrows mark intact region; triangles mark area of disrupted region) and thickened fibrointima (asterisk).