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Arrhythmogenic Ventricular Cardiomyopathy Associated With Fibromuscular Dysplasia of Ostial Right Main Coronary Artery

Farkašová Iannaccone, Silvia, MD, PhD*; Vasovčák, Peter, RNDr, PhD; Sopková, Dorota, MD*; Švajdler, Peter, MD; Dettmeyer, Reinhard, MD, JD§; Rampazzo, Alessandra, PhD; Farkaš, Daniel, MD, PhD

The American Journal of Forensic Medicine and Pathology: June 2019 - Volume 40 - Issue 2 - p 183–187
doi: 10.1097/PAF.0000000000000469
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In this article, we report the autopsy findings of a 23-year-old woman, who was found unconscious at home by her relatives. During the transportation to the hospital, the woman was handed over to the ambulance personnel, who were the first to provide cardiopulmonary resuscitation. In the hospital, after an hour-lasting asystole, the heart activity was restored. Prolonged cardiac arrest led to hypoxic brain injury, which resulted in a persistent coma. Examinations carried out during hospitalization detected hypokinetic interventricular septum, frequent ventricular extrasystoles and ventricular fibrillation. The patient died within 35 hours of admission to the hospital. Gross findings of the heart included a noticeable increase of the adipose tissue in the right ventricular wall, where histologically focal myocardial atrophy with focal transmural lipomatosis reaching endocardium were detected. Death was attributed to arrhythmogenic ventricular cardiomyopathy. Pathogenic variants in JUP gene and KCNH2 gene confirmed the diagnosis. Other finding of note was fibromuscular dysplasia of ostial right main coronary artery causing a significant luminal narrowing.

From the *Department of Forensic Medicine, Faculty of Medicine, Pavol Jozef Šafárik University, Košice;

PROGENET s.r.o., Bratislava;

Department of Pathology, Louis Pasteur University Hospital, Košice, Slovak Republic;

§Institute of Forensic Medicine, Gießen, Germany;

Department of Biology, University of Padua, Padua, Italy; and

Medico-Legal and Pathological-Anatomical Department of Health Care Surveillance Authority, Košice, Slovak Republic.

Manuscript received October 25, 2018; accepted January 15, 2019.

This case report was not funded by any outside source.

The authors report no conflict of interest.

Reprints: Dorota Sopková, MD, Department of Forensic Medicine, Faculty of Medicine, Pavol Jozef Šafárik University, Trieda SNP 1, 040 11 Košice, Slovak Republic. E-mail: d.sopkova@gmail.com.

© 2019 by Lippincott Williams & Wilkins.