Fibromuscular dysplasia of coronary arteries as a rare cause of death

Abstract

A case of fibromuscular dysplasia of the coronary arteries in a 15-year-old boy is reported. After a quarrel involving no violence the boy suddenly suffered from ventricular fibrillation, collapsed and was initially successfully defibrillated. After 37 days of deep unconsciousness the boy died of bronchopneumonia. The cause of the ventricular fibrillation was clarified only after histological investigations. Fibromuscular dysplasia of the coronary arteries with narrowing was found, which has very occasionally been described in the literature. However, its localization in the A-V node artery, as described here, only seems to have been observed once.

Cardiac conduction system- A detailed study in 46 cases of sudden death

“…FMD of the nodal arteries was seen affecting young and old individuals alike; in both the study by Charlton et al 3 Two cases of sudden death were seen in young individuals described by Michaud et al 8 and 1 case was described by Zack et al 9 Michaud et al 8 described FMD of the nodal artery as a lesion often entirely seen within the tunica media, including the present study. However, other degenerative changes like fibrosis and fat infiltration were seen more in the elderly in the present study, and was similar to the findings of Charlton et al 3 and Suarez-Mier et al…”

“…This study illustrated the importance of examination of the intramural coronary arteries and the nodal arteries, particularly in cases of sudden death of young people who die without any obvious cause. 8 Zach et al 9 examined the conduction system in a 12 year old boy who collapsed suddenly and died. The only abnormality detected was the extreme narrowing of the AV nodal artery with proliferation of smooth muscle cells with fibrosis of the media and partly of the intima.…”

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

“…85,89 In the forensic pathology literature, there are reported cases of histopathological findings consistent with FMD identified on postmortem examination among individuals with sudden cardiac death. [90][91][92][93] In these cases, the sinoatrial and atrioventricular nodal arteries were most commonly involved. Similarly, there are case reports of histopathological findings consistent with FMD of the coronary arteries among victims of sudden infant death syndrome.…”

Fibromuscular dysplasia of the coronary arteries: a case report and review of the literature

“…They described FMD as lesion of the middle or distal segment of the anterior circumflex branches of the left anterior descending coronary artery in seven premenopausal women. At that time, FMD of the coronary arteries had been determined as the cause of sudden cardiac death only with autopsy case reports (11)(12)(13)(14)(15)(16). James TN found nine cases of FMD involving the coronary arteries of small caliber in a retrospective study of 1000 cases of cardiac death in 30 years (17).…”