Progressive ECG changes in arrhythmogenic right ventricular disease Evidence foran evolving disease

Abstract

Electrocardiography results were used to assess diagnosis and evolution of arrhythmogenic right ventricular disease. The initial ECG presentation and long-term changes were analysed in 74 consecutive patients with symptomatic ventricular tachycardia and arrhythmogenic right ventricular disease. On first available tracings, a left axis deviation of the QRS was found in 18 patients. The QRS length in V1 was > or = 110 ms in 39 patients, an epsilon wave was present in 17, and a complete right bundle branch block in four patients. The T wave was negative in V1-V3 in 37 patients (50%). In 36 patients, long-term electrocardiographic follow-up of 9.5 +/- 3.2 years was available. During this period, ECG changes were observed in 20 patients (56%): negative T waves in 11 patients, a new left axis deviation in three, QRS enlargement in 13 (including eight right bundle branch block), right atrial hypertrophy in three, and paroxysmal or established atrial fibrillation in three. On studying all 110 ECG tracings (74 initial recordings +36 follow-up ECGs), we found a strong correlation between QRS or T wave changes and the length of follow-up after the first symptom; mean time interval between first ventricular tachycardia and ECG recording was significantly longer in patients with negative T waves in the right precordial leads, QRS enlargement, or left axis deviation, than in patients without such abnormalities. ECG abnormalities were more frequent at 10 year and 5 year follow-up than on initial tracings. A normal ECG was found in 40% of patients during the first year of follow-up, 8% at 5 years, and never later than the 6th year. In conclusion, electrocardiographic diagnosis of arrhythmogenic right ventricular disease may be difficult in the initial stage of the disease, since a normal ECG is found in up to 40% of patients. During the follow-up, progressive and characteristic ECG changes will occur. Arrhythmogenic right ventricular disease can be excluded if the ECG is found to be normal 6 years or later after a first ventricular tachycardia attack.

Prognostic Value of Radionuclide Angiography in Patients With Right Ventricular Arrhythmias

“…A history of syncope had no prognostic value, in contrast with the report of Blomström-Lundqvist et al 19 The rate of ECG abnormalities (60% of patients with RV abnormalities in the present study, and 68% in the study of Berder et al 22 ) is related to the extent of the disease at the time of the evaluation, and such abnormalities may appear during evolution of the disease. 26 In addition, we found no prognostic value of inducibility of SVT by EPS, the presence of which seems correlated with the prevalence of spontaneous SVT. 20,22 The same conclusion applies to the presence of late potentials at SA-ECG that have no predictive value for mortality.…”

Clinical Role of Atrial Arrhythmias in Patients With Arrhythmogenic Right Ventricular Dysplasia

“…25 Previous groups have reported a prevalence rate between 4% and 42%. 5,8-10, 26 Early small-scale studies suggested that the presence of tricuspid regurgitation and atrial enlargement were associated with the development of atrial arrhythmias. A recent large-scale retrospective study has mainly confirmed those findings.…”

Task Force on Sudden Cardiac Death of the European Society of Cardiology

“…The disease manifests in adolescents or young adults with ventricular arrhythmias [305] while clinical presentation in children is rare or underreported. The initial presenting symptom may be syncope (29%) or cardiac arrest (7-23%) [306][307][308] .…”

“…The initial presenting symptom may be syncope (29%) or cardiac arrest (7-23%) [306][307][308] . Monomorphic ventricular tachycardia with left bundle branch block morphology is the most common (70-92%) arrhythmia observed in RVC, but these may vary from asymptomatic premature ventricular beats to poorly tolerated polymorphic ventricular tachycardia [305,307,309,310] . Like hypertrophic cardiomyopathy, RVC may lead to cardiac failure [308] , but it is more frequently associated with SCD [301,311,312] and if this can be prevented, life expectancy may be normal or near normal.…”

“…Atrioventricular conduction disturbances are rare in patients with RVC [305,311] . The type of ventricular arrhythmia does not seem to be predictive of the occurrence of SCD [312] .…”