Dataset Information

Prevalence and characteristics of the Brugada electrocardiogram pattern in patients with arrhythmogenic right ventricular cardiomyopathy.

ABSTRACT:

Background

Despite distinct pathophysiology, arrhythmogenic right ventricular cardiomyopathy (ARVC) and Brugada syndrome (BrS) exhibit overlapping phenotypes. We investigated the prevalence and characteristics of the Brugada electrocardiogram (ECG) pattern in ARVC patients.

Methods

A total of 114 ARVC patients fulfilling the revised Task Force Criteria were enrolled. The Brugada ECG pattern was evaluated according to the consensus report on right precordial leads, and 1141 ECGs (median, 1; interquartile range, 1-16 ECGs/patient) were analyzed.

Results

Five patients (4%) showed a Brugada ECG pattern, which disappeared in four patients with ECGs recorded more than 2 years afterward. ARVC patients with the Brugada ECG pattern had a longer PQ interval (220 ± 62 ms vs 180 ± 35 ms, P = .02) and longer QRS duration (138 ± 25 ms vs 102 ± 23 ms, P < .001) than patients without the pattern. During follow-up (median, 11.4; interquartile range, 5.5-17.1 years), 19 ARVC patients experienced cardiac death and 29 experienced heart failure (HF) hospitalization. Kaplan-Meier analysis determined that the Brugada ECG pattern increased the risk of cardiac death and HF hospitalization (log-rank; P < .001, P < .001 respectively). The mean J-point and S-wave amplitudes of the Brugada ECG pattern were 0.29 ± 0.05 mV and 0.34 ± 0.21 mV, respectively, which were significantly lower than those of 26 age-matched BrS patients with a previous ventricular fibrillation episode (0.66 ± 0.33 mV, P < .001 and 0.67 ± 0.39 mV, P = .02 respectively).

Conclusion

The Brugada ECG pattern was infrequently encountered, was transient in ARVC patients, and was associated with a longer PQ interval, longer QRS duration, and cardiac events.

SUBMITTER: Ueda N

PROVIDER: S-EPMC8485808 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient's sudden cardiac death risk is sufficient to justify placement of an implantable cardioverter-defibrillator. The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people and that sudden death may be the first manifestation of the disease. This decision is particularly important because these are often young patients who are expected to live for many years. Although an implantable cardioverter-defibrillator can save lives in individuals with this disease, it is also well recognized that implantable cardioverter-defibrillator therapy is associated with both short- and long-term complications. Decisions about the placement of an implantable cardioverter-defibrillator are based on an estimate of a patient's risk of sudden cardiac death, as well as their preferences and values. The primary purpose of this article is to provide a review of the literature that concerns risk stratification in patients with ARVC and to place this literature in the framework of the 3 authors' considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include electric instability, including the frequency of premature ventricular contractions and sustained ventricular arrhythmia; proband status; extent of structural disease; cardiac syncope; male sex; the presence of multiple mutations or a mutation in TMEM43; and the patient's willingness to restrict exercise and to eliminate participation in competitive or endurance exercise.