Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Endowed Professor
Language English
Title Arrhythmogenic Right Ventricular Cardiomyopathy with a Desmoplakin Mutation That Appeared after Occlusion of an Atrial Septal Defect
Conference The 81st Annual Scientific Meeting of the Japanese Circulation Society (JCS2017)
Promoters Japanese Circulation Society
Conference Type Nationwide Conferences
Presentation Type Speech
Lecture Type General
Publisher and common publisher◎YOSHIDA Ayano, WATANABE Erisa, SUZUKI Atsushi, SERIZAWA Naoki, SUZUKI Tsuyoshi, SHIGA Tsuyoshi, SHODA Morio, YOSHIZAWA Saeko, UTO Kenta, HAGIWARA Nobuhisa
Date 2017/03/17
(city and name of the country)
Kanazawa, JAPAN
Society abstract PROGRAM JCS 2017 199
Summary We report the case of a 67-year-old man with arrhythmogenic right ventricular cardiomyopathy (ARVC) in whom a desmoplakin mutation appeared after occlusion of an atrial septal defect (ASD). He underwent patch closure for ASD at age 54 years. One year later, he underwent catheter ablation for atrial flutter. His left ventricular ejection fraction (LVEF) was 60%. At 58 years of age, he underwent catheter ablation for multifocal atrial tachycardia. Because he had frequent non-sustained ventricular tachycardia (VT) and inducible sustained monomorphic VT, a cardioverter-defibrillator was implanted. When he was admitted for worsening heart failure at 61 years of age, a desmoplakin mutation was detected. Because of the right ventricular dysfunction, he was diagnosed with ARVC. Although he was treated with pimobendan and tolvaptan, he was hospitalized repeatedly for heart failure and decreasing LVEF. When he was 67 years old, he experienced worsening heart failure from volume overload resistant to diuretic medication, and he began dialysis therapy. After 4 months of dialysis, he was admitted for shortness of breath and decreased blood pressure. Dialysis control was used to adjust his volume, but his pleural effusion increased. He died 32 days after admission. At autopsy, a large amount of fibrosis of the right and left ventricular myocardium was observed, in addition to an expanded right ventricle. Along with the influence of the right ventricular dysfunction caused by ASD, it was suggested that the left ventricular dysfunction was due to the ARVC with desmoplakin mutation. Various states of ARVC should be carefully assessed in order to develop an appropriate treatment strategy for ARVC.