Fibromuscular dysplasia of small coronary arteries and fibrosis in the basilar ventricular septum in mitral valve prolapse

doi:10.1016/S0002-8703(97)70136-4

American Heart Journal

Volume 134, Issue 2, August 1997, Pages 282-291

https://doi.org/10.1016/S0002-8703(97)70136-4 Get rights and content

Abstract

The mechanism of sudden cardiac death in patients with mitral valve prolapse is poorly understood. Twenty-four hearts from patients with mitral valve prolapse who suddenly died (mean age 34 ± 8 years) and 16 trauma control hearts (mean age 30 ± 7 years) were histologically studied. Dysplasia of the atrioventricular nodal artery was present in 18 of 24 hearts with mitral valve prolapse and four of 16 controls hearts ( p = 0.003). The degree of luminal narrowing, as morphometrically measured, was significantly greater in hearts with mitral valve prolapse ( p = 0.003). The degree of fibrosis in the base of the ventricular septum, as calculated by computerized morphometry, was greater in hearts with mitral valve prolapse ( p = 0.0002) and independent of age, sex, and heart weight ( p = 0.005). We conclude that arterial dysplasia in mitral valve prolapse may contribute to sudden cardiac death mediated by ventricular fibrosis. (AM Heart J 1997;134:282-91.)

Section snippets

Case selection and tissue sampling

Forty hearts were retrospectively studied after routine fixation in formalin. SCD occurred in the setting of mitral valve prolapse in 24 cases (Table I) . SCD was determined by complete autopsy examination to exclude other possible causes of death, a toxicologic examination of body fluids, and investigation of the scene of death. SCD was defined as witnessed sudden unexpected death within 6 hours of the onset of symptoms from a stable medical condition or death of an individual who had been

Age and sex

The mean age of mitral valve prolapse was 34 ± 8 years, and the mean age of control cases was 30 ± 7 years. Nine men and 15 women died from mitral valve prolapse, and 12 men and four women represented controls.

Circumstances of death

In mitral valve prolapse, three of 24 deaths occurred during exercise, 16 were witnessed arrests at rest or at sedentary activity, and five were unwitnessed during presumed sedentary activities. Five patients had a previous history of mitral valve prolapse, all of whom had palpitations or

Previous autopsy studies of mitral valve prolapse and SCD

Autopsy studies have provided several theories for the causation of SCD in patients with mitral valve prolapse and competent mitral valves. Endocardial friction lesions resulting in ventricular arrhythmias, ¹⁵ traction of an abnormally inserted valve on the conduction system, ¹⁶ and deposition of proteoglycans within the autonomic nerve supply to heart ¹³ have all been described in hearts from patients with mitral valve prolapse.

Combining data from three autopsy series of mitral valve prolapse,

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Citation Excerpt :
While the wall thickening of the sinus node artery could be observed mainly in the media, which had an increase of smooth muscle cells, the proliferations seen in the AV-node artery were mainly in the intima and usually fibrous. 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 [1–13]. Cardiac arrhythmia due to ischemia is proposed to be the lethal pathomechanism [7,10,16].
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.
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.
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.
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.

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^☆: From the Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, and the Baltimore Office of the Chief Medical Examiner.

^☆☆: The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or reflecting the views of the Department of the Army, the Department of the Air Force, or the Department of Defense.

^★: Reprint requests: Renu Virmani, MD, Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.

^★★: 0002-8703/97/$5.00 + 0 4/1/82546

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Fibromuscular dysplasia of small coronary arteries and fibrosis in the basilar ventricular septum in mitral valve prolapse☆,☆☆,★,★★

Abstract

Section snippets

Case selection and tissue sampling

Age and sex

Circumstances of death

Previous autopsy studies of mitral valve prolapse and SCD

Am Heart J

Am Heart J

Am Heart J

J Am Coll Cardiol

Am Heart J

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Hum Pathol

Am J Cardiol

J Am Coll Cardiol

Am Heart J

Am J Med

Am Heart J