Ventricular tachycardia of left bundle branch block configuration in patients with isolated right ventricular dilatation. Clinical and electrophysiological features.

Abstract

SUMMARY Electrophysiological studies showed ventricular tachycardia in five patients (four male, one female) with isolated right ventricular dilatation. All had been asymptomatic before the onset of palpitation which had developed in adolescence or early adult life. Tachycardia had been associated with syncope in four patients, and three had been resuscitated from ventricular fibrillation before investigation. The electrocardiogram during ventricular tachycardia showed a left bundle branch block pattem, and endocardial mapping at electrophysiological study confirmed the right ventricular origin. The presenting tachycardia could be induced in all patients by programmed stimulation, and in three patients ventricular tachycardia of differing configuration could be induced, but the right ventricular origin and left bundle branch block pattern were maintained. In two patients ventricular tachycardia degenerated into ventricular fibrillation. Cineangiography, cross sectional echocardiography, and multigated radionuclide angiography confirmed the dilated abnormal right ventricle while indicating that left ventricular function was normal. On resting electrocardiograms T wave inversion over the right precordial leads was the sole abnormality. There were no signs of right heart failure and exercise tolerance was normal. Four patients have received maintenance treatment with antiarrhythmic drugs, and one had undergone operative mapping and ablative surgery.Thus ventricular tachycardia complicating right ventricular dilatation may be associated with serious symptoms and ventricular electrical instability; and in adults it may be suspected on clinical grounds by inverted T waves in the right precordial leads.

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

“…28,29 At the other extreme of the spectrum are patients in whom the diagnosis of ARVD/C was not recognized at onset of symptoms but who present years later with congestive heart failure with or without ventricular arrhythmias and are often wrongly diagnosed as having dilated cardiomyopathy. 1,4,5,9,12,30 The high incidence of ARVD/C in Northern Italy has not been confirmed in studies from North America. 19 Prevalence of the disease observed in different parts of the world could be due either to clustering of the disease in some geographic areas or, more likely, to the fact that this entity is being underdiagnosed both pathologically and clinically.…”

“…[1][2][3][4][5][6][7] Clinical presentation of ARVD/C usually consists of arrhythmias of RV origin that range from isolated premature ventricular beats to sustained ventricular tachycardia or ventricular fibrillation that leads to sudden death. 1,[3][4][5][6][7][8][9][10][11] Other clinical manifestations include global or regional dysfunction and structural alterations of the RV; ECG depolarization/repolarization changes, characteristically in right precordial leads; and evolution to right or biventricular heart failure that mimics dilated cardiomyopathy. 1,[3][4][5][6][7]9,12 In the 22 years since ARVD/C was first described, 13 previously reported as auricularization of the RV, 14 considerable progress has been made in our understanding of the pathogenesis, morbid anatomy, and clinical presentation of this condition.…”

“…1,[3][4][5][6][7][8][9][10][11] Other clinical manifestations include global or regional dysfunction and structural alterations of the RV; ECG depolarization/repolarization changes, characteristically in right precordial leads; and evolution to right or biventricular heart failure that mimics dilated cardiomyopathy. 1,[3][4][5][6][7]9,12 In the 22 years since ARVD/C was first described, 13 previously reported as auricularization of the RV, 14 considerable progress has been made in our understanding of the pathogenesis, morbid anatomy, and clinical presentation of this condition. 6,7,15,16 However, a great lack of information still exists with regard to genetics, clinical diagnosis, natural history, risk stratification, outcome, and comparative efficacy of antiarrhythmic treatment and prophylaxis of lifethreatening ventricular arrhythmias and prevention of death in patients with ARVD/C.…”

Acquired fontan paradox in isolated right ventricular cardiomyopathy

“…Isolated right ventricular (RV) cardiomyopathy in itself is also quite uncommon and is a diagnosis of exclusion. [ 4 5 6 ] There is a definite male preponderance. These cases present as heart failure/syncope and features of ventricular tachycardia or left bundle branch block on electrocardiography.…”

Clinical Profile and Prognosis of Patients with Right Ventricular Dilated Cardiomyopathy: Results of a Prospective Study

“…Besides the few reports which had been mentioned casually, there are only a few documented cases in the literature. [6][7][8][9].The severity of the problem is heightened by a significant limitation of publications related to the clinical course and prognosis precisely of RV-DCM.…”