Original ArticleFibromuscular dysplasia of cardiac conduction system arteries in traumatic and nonnatural sudden death victims aged 0 to 40 years: a histological analysis of 100 cases
Introduction
In several reports, luminal narrowing by fibromuscular dysplasia of the sinus and/or AV-node artery was found in examinations of unexplained deaths and, in many cases, was considered as the cause of death [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13]. These cases were aged between 0 and 49 years; both sexes were affected [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13]. However, there are no data on the frequency of fibromuscular proliferations of arterial walls in heart-healthy control groups.
Section snippets
Materials and methods
The present histological analysis included 100 hearts selected out of nonnatural deaths examined between 1995 and 2012 in the Institute of Legal Medicine at the Rostock University Medical Center. The use of these autopsy cases for scientific investigations was approved by the Ethics Committee of the University of Rostock, Germany.
The age of the deceased ranged from 0 to 40 years (mean age, 24.4 years). Seventy-five of these cases were male, and 25 were female. The postmortem interval did not
Sinus node artery
Fifty-two out of 100 cases revealed microscopic detectable fibromuscular proliferations of the sinus node arterial wall. The media was more frequently affected (n=42) than the intima (n=16). In six cases, both the intima and media were involved.
While in the intima mild proliferation of the walls could be observed in 14 cases, moderate proliferation in 2 cases and no severe proliferation, the media showed mild findings in 36 cases, moderate findings in 4 cases, und severe findings in 2 cases (
Discussion
In numerous case reports since 1967, histological evidence of fibromuscular proliferations of arteries of the cardiac conduction system has been suspected as the cause of death when the autopsy and additional investigations could not determine another cause [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13]. Cardiac arrhythmia due to ischemia is proposed to be the lethal pathomechanism [7], [10], [16].
While there is a uniform nomenclature for fibromuscular dysplasia as a
Acknowledgments
The help of Claire Delbridge in correcting the manuscript is highly appreciated. We thank Ms. Höffer, Institute of Pathology, Rostock University Medical Center, Germany, for her excellent technical assistance.
References (19)
- et al.
Sudden death from occlusive disease of the atrioventricular node artery
Pathology
(1981) - et al.
Nonatherosclerotic narrowing of the atrioventricular node artery and sudden death
J Am Coll Cardiol
(1993) - et al.
Sudden death in young athletes
Ann Intern Med
(1967) - et al.
Occlusive lesions of cardiac conducting tissue arteries in sudden infant death syndrome
Pediatrics
(1982) - et al.
Sudden death due to fibromuscular dysplasia of the sinoatrial nodal artery
J Ky Med Assoc
(1989) Zum forensischen Stellenwert von Läsionen des Sinusknotens bei unklaren plötzlichen Todesfällen
Z Rechtsmed
(1989)- et al.
Fibromuscular dysplasia of small coronary arteries and fibrosis in the basilar ventricular septum in mitral valve prolapse
Am Heart J
(1991) - et al.
Fibromuscular dysplasia of coronary arteries as a rare cause of death
Int J Legal Med
(1996) - et al.
Sudden death caused by stricture of the sinus node artery
Am J Forensic Med Pathol
(1997)
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