Clinical Features of Noncompaction Cardiomyopathy Across All Age Strata

Abstract

Abstract Background Noncompaction cardiomyopathy (NCM) stands as a rare hereditary cardiac disorder intricately linked to adverse cardiovascular events, including heart failure, arrhythmia, and sudden cardiac death. Presently, the clinical features of NCM lack substantial support from extensive samples spanning diverse age groups. This study endeavors to systematically elucidate the clinical characteristics of NCM patients across various age demographics. Methods We retrospectively analyzed 415 patients diagnosed with NCM treated at the Guangdong Institute of Cardiovascular Disease between January 2013 and January 2023. Comprehensive datasets, encompassing clinical information, arrhythmia characteristics, as well as echocardiography and cardiac magnetic resonance imaging (CMR) data, were meticulously collected for detailed investigation and analysis. Results We stratified a cohort of 415 patients into distinct age groups: 0–1 years old (169 patients), 1–18 years old (149 patients), and > 18 years old (97 patients). Heart failure emerged as the predominant symptom across the entire population, affecting 112 (27%) patients. Specifically, heart failure constituted the prevailing clinical manifestation in patients aged > 18 years (53 patients, 54.6%), while murmur was the most observed symptom in patients aged 0–1 years and 1–18 years (33 patients, 19.5%, and 26 patients, 17.4%, respectively). In all age groups, when compared to NCM patients devoid of valvular disease, those with mitral regurgitation (MR) exhibited significantly reduced left ventricular ejection fraction (LVEF) and left ventricular fractional shortening (FS) (p < 0.05). Additionally, left ventricular end-diastolic diameter (LVESD) and left ventricular end-systolic diameter (LVEDD) were significantly increased in NCM patients with MR (p < 0.05). Furthermore, there was a significant correlation between LVESD diameter and LVEDD diameter with MR area in NCM patients (p < 0.05). Conclusion Across all age strata, individuals with NCM and concomitant MR consistently manifested left ventricular (LV) dilatation and LV systolic function impairment. There were significant differences in clinical symptoms, arrhythmia, CHD and valvular heart disease among distinct age groups. Recognizing the paramount significance of age-related clinical characteristics is imperative for accurate diagnosis, effective treatment, and robust research initiatives concerning NCM patients.