Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

• DOI: 10.20944/preprints202106.0276.v1
• Publication Date: 2021-06-10T06:58:31Z
• Authors: Agata Paszkowska; Alicja Mirecka-Rola; Dorota Piekutowsk...; Elżbieta Ciara; Łukasz Mazurkiewicz; Katarzyna Biegano...; Lidia Ziółkowska
• Keywords: Left ventricular noncompaction; Etiology; Atrioventricular block; Sinus bradycardia

Background: Left ventricular noncompaction (LVNC) is a genetically determined cardiomyopathy, that occurs following disruption of endomyocardial morphogenesis. The purpose this study was to identify the clinical characteristics and genetic profile children with LVNC. Methods: From February 2008 July 2020, total 32 (median 11.5 years) LVNC were prospectively enrolled followed up for median 4.02 years. Diagnosis made based on characteristic features in echocardiography cardiovascular magnetic resonance (CMR). Patients’ symptoms, family history, ECG, Holter ECG tests also evaluated. Results: most common presenting symptom heart failure (31% children). abnormalities noted 56% patients. prominent arrhythmias, sinus bradycardia paroxysmal third-degree atrioventricular block. Most patients (94%) met criteria CMR confirmed diagnosis 82% cases. molecular etiology found 53% children. Conclusion: Although arrhythmias very frequent our group, thromboembolic events syndromes rare. For accurate reliable assessment LVNC, it necessary get know their history detailed profile.