Primary Electrical Disorder or Primary Cardiomyopathy?
Author(s) -
Zsófia Szentpáli,
Tamás Szili-Török,
Kadir Çalişkan
Publication year - 2013
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.112.144949
Subject(s) - medicine , cardiomyopathy , catecholaminergic polymorphic ventricular tachycardia , erasmus+ , restrictive cardiomyopathy , brugada syndrome , cardiology , heart failure , ryanodine receptor 2 , ryanodine receptor , art , the renaissance , art history , calcium
Noncompaction cardiomyopathy (NCCM) is a rare primary cardiomyopathy caused by an arrest in myocardial morphogenesis.1 The clinical manifestations are highly variable from asymptomatic to severe heart failure, arrhythmias, sudden cardiac death, or systemic thrombo-embolic events. Association of NCCM and electric disorders has been reported as rare case reports with WPW syndrome and Brugada syndrome,2 But to the best of our knowledge, an association with catecholaminergic polymorphic ventricular tachycardia (CPVT) and NCCM has never been reported.The aim of the case report is to present unique features of both NCCM and gene mutation–proven CPVT in 1 patient, raising new questions.A 39-year-old woman was referred with frequent palpitations, exercise-induced dyspnea, fatigue, and tendency to faint. Her medical and family history revealed no cardiac disease. ECG demonstrated sinus bradycardia, 50 beats per minute, and mild inferolateral repolarization abnormalities. Echocardiography and MRI showed normal left ventricular function (ejection …
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