Key result
Arrhythmias in pediatric left ventricular hypertrabeculation are linked to class 4 or 5 genetic variants.
Why the study?
Distinguishing benign from pathological left ventricular hypertrabeculation (LVHT) is challenging, prompting the need to describe arrhythmic manifestations in pediatric patients and correlate them with genetic results and clinical markers.
Cohort (n=140)
No
p-value: p=0.037
In pediatric patients with left ventricular hypertrabeculation, arrhythmias are relatively common (23.6%) and significantly associated with pathogenic genetic variants.
Arrhythmias affect nearly one-quarter of children with LVHT; hypothesis-generating and leaves open prospective risk-stratification studies.
Background. Left ventricular hypertrabeculation (LVHT) is a myocardial disorder with different clinical manifestations, from total absence of symptoms to heart failure, arrhythmias, sudden cardiac death (SCD), and thromboembolic events. It is challenging to distinguish between the benign and pathological forms of LVHT. The aim of this study was to describe the arrhythmic manifestations of LVHT in a large group of pediatric patients and to correlate them with genetic results or other clinical markers. Methods. We retrospectively enrolled 140 pediatric patients with diagnosis of LVHT followed at our Institution from 2013 to 2023. Data regarding family history, instrumental exams, cardiac magnetic resonance, genetic testing and outcomes were collected. Most of them had isolated LVHT (80.7%); in other patients, mixed phenotypes (hypertrophic or dilated cardiomyopathy or congenital heart disease) were present. Results. Arrhythmias were found in 33 children (23.6%): 13 (9.3%) supraventricular tachyarrhythmias; 14 (10%) ventricular arrhythmias (five frequent PVCs (premature ventricular contractions), eight patients with ventricular tachycardia (VT), one ventricular fibrillation (VF)); two (1.4%) sinus node disfunctions; two (1.4%) complete atrio-ventricular blocks (AVB), three (2.1%) paroxysmal complete AVB, one (0.7%) severe I degree AVB. Three patients received an ICD (implantable cardioverter defibrillator). Comparison between LVHT patients with (33 pts) and without (107 pts) arrhythmias as regards genetic testing showed a statistical significance for the presence of class 4 or 5 genetic variants and arrhythmic manifestation (p = 0.037). Conclusions. In our pediatric cohort with LVHT, good outcomes were observed, but arrhythmias were not so rare (23.6%); no SCD occurred.
No takes yet. Share an insight, caveat, or question.
Battipaglia et al. (2024) conducted a cohort in Left ventricular hypertrabeculation (LVHT) (n=140). Arrhythmias vs. No arrhythmias was evaluated on Presence of class 4 or 5 genetic variants (p=0.037). In pediatric patients with left ventricular hypertrabeculation, the presence of arrhythmias was significantly associated with class 4 or 5 genetic variants (p=0.037).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: