Clinical profile of arrhythmogenic right ventricular cardiomyopathy in Chinese patients.
OBJECTIVE
To study the clinical profile of Chinese patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).


METHODS
Chinese patients who fulfilled the diagnostic criteria of ARVC proposed by the Task Force of the European Society of Cardiology and of the scientific council on cardiomyopathy of the International Society and Federation of Cardiology were recruited for analysis.


METHODS
Clinical data of patients with ARVC including age, sex, family history, presenting symptoms, electrocardiograph (ECG), echocardiography, cardiac catheterization, magnetic resonance imaging (MRI), electrophysiology study (EPS) and therapeutic intervention were analyzed.


RESULTS
Eleven patients (seven males) were diagnosed with ARVC. Mean age at clinical presentation was 42.6+/-14.8 years. Two patients (18.1%) had positive family history of ARVC or premature sudden cardiac death. The commonest presenting symptoms were palpitation (73%) and dizziness (46%). Spontaneous ventricular tachycardia (VT) was the presenting arrhythmia in 54% and 1 (9%) with ventricular fibrillation and cardiac arrest. Seven patients (64%) had the ECG abnormality as defined by the Task Force. Echocardiography showed right ventricular (RV) dilatation in five patients (46%) and all patients had normal left ventricular function. Nine patients (90%) had RV wall thinning or fibrofatty replacement on MRI examination. Inducible monomorphic VT was detected in four out of nine patients at EPS. All eight patients had normal coronary arteries and left ventriculogram but RV dilatation and global hypokinesia was seen in three patients. Implantable cardioverter defibrillators were implanted in five patients and two of them had shocks delivered during the follow-up period.


CONCLUSIONS
In this study, familial incidence of premature sudden death in patients with ARVC appears to be low and left ventricular involvement in affected individuals is uncommon. MRI is still the best investigation for ARVC.
Fung
W H
WH
Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong, Hong Kong, China.
Sanderson
J E
JE
D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000503:physiopathology* / Q000628:therapy; D044466:Asian People; D006328:Cardiac Catheterization; D015331:Cohort Studies; D017023:Coronary Angiography; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015635:Radionuclide Ventriculography; D012189:Retrospective Studies; D016896:Treatment Outcome; D016276:Ventricular Function / Q000502:physiology