Elsevier

Cardiovascular Pathology

Volume 23, Issue 1, January–February 2014, Pages 12-16
Cardiovascular Pathology

Original Article
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

https://doi.org/10.1016/j.carpath.2013.07.001Get rights and content

Abstract

Background

Since 1967, numerous case reports have described fibromuscular alterations of the sinus node artery and/or the atrioventricular node artery as a potential cause of death. However, the prevalence of these changes in a healthy population has only rarely been investigated systematically.

Methods

The arteries of the cardiac conduction system were studied systematically, by means of routine histology, in 100 cases of victims aged 0 to 40 years with a nonnatural cause of death.

Results

Microscopic alterations were seen in the walls of sinus node arteries in 52 out of 100 cases, in the walls of atrioventricular node arteries in 63/100 cases, and in the walls of small vessels in 60/100 cases.

Conclusions

The results demonstrate that microscopically detectable findings of the cardiac conduction system arteries similar to fibromuscular dysplasia do not indicate a defined disease and should not be considered as a cause of death when there are no macroscopic findings in the coronary arteries.

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.

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