Fibromuscular dysplasia of cardiac conduction system arteries in traumatic and nonnatural sudden death victims aged 0 to 40 years: a histological analysis of 100 cases

First International Consensus on the diagnosis and management of fibromuscular dysplasia

“…atrioventricular and sinoatrial nodal arteries). [177][178][179][180][181][182][183][184][185][186][187] Both have been associated with sudden cardiac death in rare post mortem reports in adults and children. However, although histopathologically consistent with FMD, these patterns of disease appear distinct from the clinical phenotype of both 'typical' SCAD and 'classical' renal and/or cerebrovascular multifocal FMD.…”

Recent Developments in the Understanding and Management of Fibromuscular Dysplasia

“…28 However, Zack and colleagues investigated the incidence of histopathologically confirmed FMD of the nodal arteries in patients who sustained non–cardiac sudden death (eg, accidents, suicides) and found microscopic alterations of the sinus node arteries in 52 of 100 cases, atrioventricular nodal arteries in 63 of 100 cases, and 60 of 100 small vessels consistent with FMD. 30 Therefore, it seems likely that histopathologic findings of the nodal arteries mimicking FMD seen in other vascular territories is a potential incidental autopsy finding and is likely disease causing. It is our opinion and those of the authors of the AHA Scientific Statement these pathological findings do not represent the clinical disease process of FMD discussed in this manuscript.…”

Dissecting the Cardiac Conduction System: Is It Worthwhile?

“…Previous studies that have focused on determining if fibromuscular dysplasia of cardiac CS arteries can be regarded as the cause of death led to conflicting results. 7,8 In the manuscript by Zack et al, 7 a high percentage of nonnatural sudden death victims demonstrated fibromuscular dysplasia of cardiac CS arteries; in the vast majority of their cases, the changes were mild and accompanied by microscopically detectable proliferations of small intramural coronary arteries. Cohle et al 8 concluded from their study that narrowing of the AV node artery by fibromuscular hyperplasia was commonly encountered in patients with sudden death and was considered lethal.…”