Abstract
Abstract Left ventricular non-compaction (LVNC) represents a genetically heterogeneous primary cardiomyopathy which occurs in both children and adults, can be isolated or combined with other congenital heart diseases and reveals substantial clinical polymorphism. Isolated LVNC is a rare phenomenon more commonly being a part other cardiovascular phenotypes such as hypertrophic cardiomyopathy, restrictive cardiomyopathy or congenital heart disorders. Clinical features on LVNC include main triade: heart failure, ventricular arrhythmias (VA) and thromboembolism. Purpose The aim of this study was to analyze the clinical characteristics of arrhythmias in children with LVNC. Methods We enrolled 15 patients diagnosed with LVNC. For all patients enrolled the clinical examination included ECG, echocardiography, Holter monitoring, family history and neurological examination. The echocardiographic diagnosis for LVNC was based on the criteria of Stollberger and Jenni. Several patients additionally received MRI. Target sequencing and WES were performed using a targeted panel of 108 cardiomyopathy-associated genes. All patients had a written parental consent form for including in this study. Results The mean age oat presentation was 7.46±4.4 year (from 0.5 to 14 year old). 4 patients had ventricular arrhythmias, which manifested as isolated premature ventricular contractions (PVCs) in two patients; ventricular tachycardia (VT) in two patients, 3 – supraventricular arrhythmias, 2 – WPW syndrome/phenomenon, 5 - atrioventricular block (1 and 2 degrees), atrial fibrillation – 1. Antiarrhythmic therapy received 7 patients (3 with VA; 3 with SVA). In total 21 variants of interest were identified in all 15 patients in 18 different genes. All four patients with variants in ion channel genes had documented supraventricular and ventricular arrhythmias. In 3 out of 5 patients with AV block variants in contractile sarcomeric and related genes were detected. Four patients reached endpoints such as cardiovascular death (2 patients) and heart transplantation (2 patients). Conclusions Arrhythmic manifestations in children with left ventricular non-compaction can vary from conduction disease to SVT and ventricular arrhythmias and these arrhythmias have been associated with prognosis and outcome. Funding Acknowledgement Type of funding source: None
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