Retrospective evaluation of patients with left ventricle noncompaction cardiomyopathy at our pediatric cardiology department

Noncompaction cardiomyopathy (NCKMP) is defined as a rare cardiomyopathy characterized by the stagnation of the intrauterine development process of the myocardium and the persistence of the evident ventricular trabecular structure seen in the early period of embryogenesis in the postnatal and adult period. In this study, fifty patients who were followed up between the dates October 1st, 2014 and October 1st, 2019 with the diagnosis of left ventricular noncompaction cardiomyopathy in the ward and outpatient clinics of Bursa Uludağ University Faculty of Medicine, Department of Pediatrics, Pediatric Cardiology department were screened. The age of diagnosis, gender distribution, presentation symptoms, etiological distribution, presence of accompanying diseases, echocardiography findings, holter and angiography results, prognosis, medical and interventional treatments, duration of the treatment and the patients' response to the treatment were evaluated. Fifty-four percent (n:27) of the cases constituting the study group were male, while the remaining 46% (n:23) were female. The median age of was computed as 7 months (0.1-180 months). Eight percent of the cases (n:4) were asymptomatic. Among the patients with at least one symptom, the most common presenting symptom was murmur, seen in 68% of the cases (n:34). Another unaccompanied symptoms were respiratory distress, seen in 38% of the cases (n:19); and cardiomegaly, seen in 28% of the cases (n:14). The frequency of asymptomatic patients was higher in patients over 2 years old (p = 0.032). The median follow-up period of the patients was 47 months, with a minimum of 1 month and a maximum of 156 months. The number of patients with ongoing disease was 46 (92%), and 4 (8%) of the patients in the study have passed away. As a result; the data obtained from our study is important in terms of guiding the diagnosis, follow-up and treatment processes of patients with NCKMP. Echocardiography is the most important diagnostic method in the follow-up of these patients. Nowadays, owing to its more widespread use, NCKMP can be diagnosed earlier and with early treatment, clinical pictures such as heart failure (HF), thromboembolism, ventricular arrhythmia and sudden cardiac death can be prevented.