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Published on: February 8, 2022
Intramural coronary artery dysplasia of the ventricular septum and sudden death
1Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Insights
Sudden cardiac death in young adults can be caused by intramural coronary artery dysplasia, a small vessel disease affecting the ventricular septum. This condition, marked by artery thickening and narrowing, may occur without prior heart issues or family history.
Area of Science:
- Cardiovascular Pathology
- Sudden Cardiac Death Etiology
- Coronary Artery Disease
Background:
- Sudden unexpected death in young adults is a critical concern.
- Identifying the underlying causes is crucial for prevention and understanding.
- Previous cardiac history or family history is often absent in these cases.
Observation:
- Four cases of sudden unexpected death in individuals aged 12-31 years were analyzed.
- Death occurred during exercise in three of the four cases.
- Autopsies revealed marked intramural coronary artery dysplasia of the ventricular septum.
Findings:
- The coronary artery dysplasia showed severe medial thickening, smooth muscle cell disorganization, and luminal narrowing.
- Myocardial fibrosis was present in three of the four cases.
- No evidence of hypertrophic cardiomyopathy or other significant cardiac/extracardiac lesions was found, except for an atrial septal defect in one case.
Implications:
- Intramural coronary artery disease of the ventricular septum can be an isolated cause of sudden cardiac death in young adults.
- This finding highlights the importance of investigating small vessel coronary abnormalities.
- Understanding these specific pathologies may aid in risk stratification and prevention strategies for sudden cardiac death.
Abstract:
We report four cases of sudden unexpected death in three males and one female aged 12 to 31 years. Death occurred during exercise in three of four cases, and there was no history of sudden death or previous cardiac history in any patient. At autopsy, there was marked intramural coronary artery dysplasia of the ventricular septum, accompanied in three of the four cases by myocardial fibrosis. The arterial dysplasia was characterized by severe medial thickening with smooth muscle cell disorganization and marked luminal narrowing. There was no evidence of myofiber disarray or asymmetric septal hypertrophy to suggest hypertrophic cardiomyopathy. Other than an ostium secundum type atrial septal defect in one case, there were no associated cardiac or extracardiac lesions found at complete autopsy of these individuals. We conclude that small vessel disease of intramural coronary arteries of the ventricular septum may be an isolated finding leading to sudden cardiac death in young adults.
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