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Intravascular Papillary Endothelial Hyperplasia in the Coronary Artery: An Unusual Cause of Massive Myocardial Infarction in Hypoplastic Left Heart Syndrome

Raya Safa, Richard Garcia, Ralph Delius, Gunjanpreet Kaur, Lara Youssef, Janet Poulik, Bahig M Shehata
Fetal and Pediatric Pathology 2019 May 14, : 1-7

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that usually involves the head and neck or extremities. Involvement of the coronary arterial system is unreported.

CASE: A 1-month-old patient born with hypoplastic left heart syndrome died from a massive myocardial infarction shortly after first stage palliation with Norwood/Sano. Autopsy demonstrated a massive univentricular hemorrhagic infarction with complete occlusion of the left main coronary artery and its intramural branches by intraluminal papillary endothelial hyperplasia (IPEH). Immunostaining with CD34 and CD31 confirmed the diagnosis. The inferior and superior mesenteric artery branches also had IPEH.

CONCLUSION: IPEH can involve the coronary vasculature, can be multifocal and can occur in the newborn.


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