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Ventricular Dysrhythmias During Long-Term Follow-Up in Patients With Inherited Cardiac Arrhythmia
Gustaf D S Sitorus1, Ahmed A Y Ragab1, Charlotte A Houck1
1Department of Cardiology, Erasmus University Medical Center, Rotterdam, the Netherlands.
Insights
Frequent ventricular arrhythmias and premature complexes are common in inherited cardiac arrhythmias (ICA). Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients showed higher rates of ventricular dysrhythmias (VDR) and malignant ventricular tachyarrhythmias (VTA), with a significant recurrence rate observed during long-term follow-up.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Inherited cardiac arrhythmias (ICA) encompass a spectrum of genetic disorders affecting the heart's electrical system.
- Previous reports have not detailed the interrelationship and incidence of various ventricular arrhythmias in different ICA types.
- Understanding these patterns is crucial for risk stratification and management.
Purpose of the Study:
- To investigate the incidence and recurrence rates of ventricular dysrhythmias (VDR) and malignant ventricular tachyarrhythmias (VTA) in patients with diverse ICAs.
- To compare the occurrence of frequent premature ventricular complexes (fVPC) and nonsustained ventricular tachycardia (nsVT) across different ICA subtypes.
- To analyze the long-term follow-up data for VDR and VTA events in a cohort of ICA patients.
Main Methods:
- A cohort of 167 patients with diagnosed ICA (ARVC, BrS, CPVT, LQTS, SQTS) underwent frequent 24-hour Holter monitoring.
- Data collected over a mean follow-up period of 4.6 years included incidence of fVPC, nsVT, sustained VT, and ventricular fibrillation (VF).
- Statistical analyses, including odds ratios (OR) and confidence intervals (CI), were used to compare arrhythmia frequencies between ICA groups.
Main Results:
- Frequent premature ventricular complexes (fVPC) and nonsustained ventricular tachycardia (nsVT) were observed in 19% and 13% of all patients, respectively.
- Patients with Brugada syndrome (BrS) and long QT syndrome (LQTS) exhibited significantly lower rates of fVPC and nsVT compared to arrhythmogenic right ventricular cardiomyopathy (ARVC).
- The overall recurrence rate for malignant ventricular tachyarrhythmias (VTA) was 33% during the follow-up period, with ARVC patients showing the highest incidence of fVPC, nsVT, and VTA.
Conclusions:
- A variety of ventricular dysrhythmias (VDR) and malignant ventricular tachyarrhythmias (VTA) occur in patients with inherited cardiac arrhythmias (ICA).
- The recurrence rate of malignant VTA is considerable, underscoring the need for vigilant monitoring in these patients.
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a higher burden of ventricular arrhythmias compared to other ICAs like BrS and LQTS.
Abstract:
Reports on development of frequent ventricular premature complexes (fVPC), (non)sustained ventricular tachycardias ([n]sVT), or ventricular fibrillation (VF) and their interrelationship in patients with different inherited cardiac arrhythmia (ICA) have sofar not been reported. The aim of this study is therefore to examine incidences and recurrences rates of sVT and VF ("malignant ventricular tachyarrhythmias, VTA") in addition to the incidence of fVPC and nsVT ("ventricular dysrhythmias, VDR") in patients with various ICA during long-term follow up. Patients (N = 167, 88 male, age 45 ± 15 years) with ICA including definite/borderline arrhythmogenic right ventricular cardiomyopathy (ARVC, N = 47), Brugada syndrome (BrS, N = 71), catecholaminergic polymorphic ventricular tachycardia (CPVT, N = 7), long QT syndrome (LQTS, N = 41) or short QT syndrome (SQTS, N = 1) who had frequent 24-hour Holter monitoring during a follow-up period of 4.6 ± 4.4 years. During the initial screening visit, 15 patients had a history of malignant VTA. fVPC and nsVT was observed in respectively 19% (OHCA/VF/sVT: N = 9) and 13% (OHCA/VF/sVT: N = 4) of all patients. Compared with the ARVC group, patients with BrS and LQTS had less frequent fVPC and nsVT (fVPC: odds ratio [OR] 0.20, 95% confidence interval [CI] 0.08 to 0.49, p <0.000 and OR 0.09, 95% CI 0.02 to 0.33, p <0.000; nsVT:OR 0.17, 95% CI 0.06 to 0.50, p = 0.001 and OR 0.09, 95% CI 0.02 to 0.46, p = 0.003). The recurrence rate of malignant VTA was 33%. In conclusion, variety of VDR and malignant VTA were found during long-term follow-up in patients with ICA. During nearly a 5 years follow-up period, the recurrence rate of malignant VTA was considerable. fVPC, nsVT, and malignant VTA were most often found in patients with an ARVC.
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