VENTRICULAR ARRHYTHMIAS DURING EXERCISE TESTING IN PEDIATRIC PATIENTS WITH ARRHYTHMOGENIC CARDIOMYOPATHY AT FIRST PRESENTATION AND WITH DIFFERENT VENTRICULAR INVOLVEMENT Correlation between arrhythmic expression and phenotype: is really possible?

IF 5.6 2区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

Heart rhythm Pub Date : 2025-03-26 DOI:10.1016/j.hrthm.2025.03.1983

Marianna Cicenia, Michele Lioncino, Nicoletta Cantarutti, Irma Battipaglia, Aurelio Secinaro, Rachele Adorisio, Anwar Baban, Massimo Stefano Silvetti, Fabrizio Drago

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引用次数: 0

Abstract

Background: Ventricular arrhythmias (VAs) may be the first clinical manifestation in pediatric patients with arrhythmogenic cardiomyopathy (ACM). The arrhythmogenicity of exercise testing (ET) remains unclear.

Objective: Describe the arrhythmogenicity of ETs in pediatric early ACM.

Methods: Pediatric patients diagnosed with definite ACM were enrolled. ET results at the first presentation and off of therapy were analyzed. Comparison with a control group [premature ventricular complexes (PVCs) without structural heart disease] was performed.

Results: At baseline, VAs were observed in 20 (80%) patients: 3 had non-sustained ventricular tachycardia (ns-VT) and 17 isolated PVCs. No PVC morphology was significantly more prevalent among ACM phenotypes. At peak exercise, VAs were present in 40% of patients and 10% of controls. During recovery, VAs occurred in 17 (68%) and 4 (8%) of controls. Polymorphism and ns-VTs during recovery were more prevalent in biventricular-ACM (BIV-ACM) than non-biventricular-ACM (p=0.03; p=0.03). Irregular VAs were present in 80% of BIV-ACM. Compared with controls, PVCs with LBBB/superior axis at baseline and VAs during recovery were significantly associated with the probability of having ACM (p=0.0038, p=<0.0001, respectively).

Conclusions: VAs behavior during ET at the beginning of ACM is highly variable and suppression during exercise is not uncommon. Nevertheless, the presence at the peak of exercise is more common in ACM patients. Baseline LBBB superior axis PVCs and VAs during recovery correlate with the presence of ACM. VAs morphology and ET behavior cannot predict ACM phenotype. Polymorphism and ns-VTs are more prevalent in patients with BIV-ACM.

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来源期刊

Heart rhythm 医学-心血管系统

CiteScore

10.50

自引率

5.50%

发文量

1465

审稿时长

24 days

期刊介绍： HeartRhythm, the official Journal of the Heart Rhythm Society and the Cardiac Electrophysiology Society, is a unique journal for fundamental discovery and clinical applicability. HeartRhythm integrates the entire cardiac electrophysiology (EP) community from basic and clinical academic researchers, private practitioners, engineers, allied professionals, industry, and trainees, all of whom are vital and interdependent members of our EP community. The Heart Rhythm Society is the international leader in science, education, and advocacy for cardiac arrhythmia professionals and patients, and the primary information resource on heart rhythm disorders. Its mission is to improve the care of patients by promoting research, education, and optimal health care policies and standards.