Arrhythmogenic right ventricular cardiomyopathy/dysplasia: Analysis based on six cases

Abstract

Background: We sought to investigate the profile of symptoms and results of investigations among six cases of suspected arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).
Methods: The diagnosis of ARVC/D was made on the basis of standardised diagnostic criteria proposed by the study group on ARVC/D of the European Society of Cardiology. A study was conducted involving six patients with suspected ARVC/D that were diagnosed and treated at our centre in the years 1992-2004.
Results: All patients presented with a typical history and with similar complaints and symptoms: limitation of exercise toleration, palpitations, dizziness, presyncope and syncope. In all six cases ECG abnormalities were detected, namely T wave inversion, prolonged QRS complexes in V1-V3 or/and epsilon waves. Echocardiographic abnormalities were also detected in all cases in the form of global or segmental dilation and a reduction in right ventricular ejection fraction, morphological irregularity of the endocardium and tricuspidal valve insufficiency. On the basis of diagnostic criteria we diagnosed ARVC/D in four cases and the borderline variant of ARVC/D in the remaining two.
Conclusions: ARVC/D is a heart muscle disease with varied and complex presentation. The profile of symptoms and the results of investigations and diagnostic procedures are varied and can assume various combinations. Accurate diagnosis can be established in most cases as a result of the non-invasive and widely-used techniques of ECG, 24-hour Holter monitoring and echocardiography. (Cardiol J 2007; 14: 396-401)