Key result
Histopathologic examination of two children who died suddenly revealed intimal fibroplasias of the right coronary artery coexisting with fatty infiltration of the right ventricular myocardium.
Case Report (n=2)
In children with sudden death, right coronary artery fibromuscular dysplasia may cause ischemic fatty infiltration of the right ventricle mimicking arrhythmogenic right ventricular dysplasia.
May mimic ARVD in pediatric sudden death; leaves open confirmation in larger series.
We report two unusual cases of sudden unexpected death in children. Histopathologic examination showed intimal fibroplasias, a variant of fibromuscular dysplasia of the right coronary artery associated in both cases with fatty infiltration of the right ventricular myocardium. The significance of this particular combination of two lesions known to induce a sudden death in young people is discussed.
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Osterheld et al. (2013) conducted a case report in Sudden unexpected death (n=2). Intimal fibroplasias of the right coronary artery and fatty infiltration of the right ventricular myocardium was evaluated on Histopathological findings at autopsy. Histopathologic examination of two children who died suddenly revealed intimal fibroplasias of the right coronary artery coexisting with fatty infiltration of the right ventricular myocardium.
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