Arrhythmogenic Ventricular Cardiomyopathy Associated With Fibromuscular Dysplasia of Ostial Right Main Coronary Artery
| Autor: | Alessandra Rampazzo, Silvia Farkašová Iannaccone, Dorota Sopková, Peter Švajdler, Reinhard B. Dettmeyer, Peter Vasovčák, Daniel Farkaš |
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| Rok vydání: | 2019 |
| Předmět: |
medicine.medical_specialty
ERG1 Potassium Channel medicine.medical_treatment Lipomatosis Autopsy Fibromuscular dysplasia Pathology and Forensic Medicine Young Adult Internal medicine medicine Humans Cardiopulmonary resuscitation Interventricular septum Asystole Endocardium Arrhythmogenic Right Ventricular Dysplasia business.industry Arrhythmias Cardiac medicine.disease medicine.anatomical_structure Death Sudden Cardiac Ventricular fibrillation Mutation cardiovascular system Cardiology Female gamma Catenin business Cardiomyopathies |
| Zdroj: | The American journal of forensic medicine and pathology. 40(2) |
| ISSN: | 1533-404X |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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