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Familial fibromuscular dysplasia presenting as sudden death.

Dominguez Fe, Tate Lg, Robinson Mj

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Abstract

Fibromuscular dysplasia involving the aorta, its main arch branches, and the coronary arteries is infrequent. Familial occurrence of arterial fibromuscular dysplasia is rarely recognized in infancy. We report three infant siblings with this disorder who presented with sudden unexpected death secondary to acute myocardial infarction as a complication of fibromuscular dysplasia involving the aorta, its main arch branches, and the major coronary arteries. This condition must be considered in the differential diagnosis of identifiable causes of the sudden infant death syndrome.

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What this paper is about

Fibromuscular dysplasia involving the aorta, its main arch branches, and the coronary arteries is infrequent. Familial occurrence of arterial fibromuscular dysplasia is rarely recognized in infancy. We report three infant siblings with this disorder who presented with sudden unexpected death secondary to acute myocardial infarction as a complication of fibromuscular dysplasia involving the aorta, its main arch branches, and the major coronary arteries. This condition must be considered in the differential diagnosis of identifiable causes of the sudden infant death syndrome.

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Available abstract

Fibromuscular dysplasia involving the aorta, its main arch branches, and the coronary arteries is infrequent. Familial occurrence of arterial fibromuscular dysplasia is rarely recognized in infancy. We report three infant siblings with this disorder who presented with sudden unexpected death secondary to acute myocardial infarction as a complication of fibromuscular dysplasia involving the aorta, its main arch branches, and the major coronary arteries. This condition must be considered in the differential diagnosis of identifiable causes of the sudden infant death syndrome.

Key concepts: Fibromuscular dysplasia, Medicine, Sudden death, Aorta, Dysplasia, Coronary arteries, Cardiology, Differential diagnosis

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