Clinical study of 39 Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.

BACKGROUND: There are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). The purpose of this study was to describe the clinical characteristics of ARVD/C patients from China, particularly to define the features of electrocardiograph and treatment outcomes. METHODS: Thirty-nine patients hospitalized in Fu Wai Cardiovascular Hospital from 1998 to 2006 were included. The data were obtained from the medical archive and the follow-up records. RESULTS: Of these patients 33 were male and 6 female (age at the first presentation was (34.9 +/- 9.8) years). The most common symptoms were palpitation (62%) and syncope (44%). Right precordial QRSd >or= 110 ms was detected in 69% of the patients, epsilon wave in 59%, and a ratio of QRSd in V(1) + V(2) + V(3)/V(4) + V(5) + V(6) >or= 1.2 in 82%. The most frequent features of electrocardiogram in patients without right bundle-branch block were T-wave inversions and S-wave upstroke in V(1)-V(3) >or= 55 ms (96% and 90% of 28 patients, respectively). Radiofrequency catheter ablation (RFCA) for ventricular tachycardia (VT) was successful in 15 (68%) of 22 patients. The recurrence rate of VT was 46% (7/15) during the follow-up of (16.7 +/- 11.2) months. Seven patients had cardioverter/defibrillator (ICD) implanted plus drug therapy and 17 patients took antiarrhythmic drugs alone. During the follow-up of (35.6 +/- 19.0) months, all patients with ICD implanted received at least one appropriate ICD shock. One patient died of ventricular fibrillation suddenly and one patient underwent heart transplantation for progressive biventricular heart failure during the drug therapy alone. CONCLUSIONS: This study demonstrated the clinical and ECG features of the 39 ARVD/C Chinese patients. ICD provided life-saving protection by effectively terminating malignant arrhythmias, and the high recurrence of VT was the major problem of RFCA therapy.

[Clinical and ECG features of arrhythmogenic right ventricular cardiomyopathy: a retrospective analysis of 31 cases].

OBJECTIVE: To retrospectively analyze the clinical and electrocardiographic features of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: The clinical, electrocardiographic features and the efficacy of various therapies were analyzed in 31 patients (27 males) diagnosed as ARVC according to the criteria established by European Society of Cardiology. RESULTS: The averaged age when the ARVC was first diagnosed was (34.7 +/- 9.4) years (19 - 58 years), palpitation was present in 28 patients (90.3%) and syncope in 13 patients (41.9%), a family history of sudden death was present in 1 patient. Dilatated right ventricle was documented in 29 patients by echocardiography and (or) magnetic resonance imaging (MRI), 2 of them with dilated left ventricles. ECG changes included: T wave inversion, mostly seen in precordial leads (100%); epsilon (epsilon) wave (54.8%); QRS duration >or= 110 ms in V(1) to V(3) (83.9%); reduced extremity amplitude (41.9%); the first degree of AV block (22.6%); sustained VT (100%) including 15 monomorphic VT (48.4%) and 16 polymorphic VT (51.6%). The mean values of QRS duration in leads of V(1 - 3) [(120.8 +/- 13.7) ms] was significantly longer than that in V(4 - 6) [(99.4 +/- 13.7) ms, P < 0.05]. Fourteen patients underwent radiofrequency catheter ablation (RFCA) with an immediate success rate of 78.6% (11/14). During follow up (18.3 +/- 10.2) months, VT reoccurred in 6 patients (54.5%). The remaining 17 patients were treated with conventional medications, 7 of them were medicated under implanted cardioverter defibrillator (ICD). During the follow-up (35.6 +/- 19.0) months, VT reoccurred in 11 patients (64.7%) and one patient died suddenly. CONCLUSIONS: ARVC patients developed symptoms at mid-30s with significant ECG changes including appearance of an epsilon wave, T wave inversion and QRS duration >or= 110 ms in leads of V(1 - 3). The long term therapy efficacy was not satisfactory both for RFCA and conventional medications and ICD implantation should be recommended to patients with ARVC.