Retrospective evaluation of patients with dilated cardiomyopathy at OUR pediatric cardiology department

Cardiomyopathies are a group of disease which are caused by different type of etiologies, characterized by myocardial involvement and mostly lead to heart failure. Incidence of pediatric cardiomyopathies in 0-18 aged children is 0,113 percent in a year. When evaluating subgroups of cardiomyopathies as 5 groups, they are classified as dilated, restrictive, hypertrophic, arrhythmogenic right ventricle dysplasia/cardiomyopathy and non-classified. Dilated cardiomyopathy (DCMP) is the most common type of cardiomyopathy in childhood and its incidence is 60 percent in all types of cardiomyopathies. In today's world, DCMP is one of the most common reason for heart failure during childhood and constitute a big part of patients who needs heart transplantation. Diagnosis is based on presentation of left or both ventricle dilatation and reduced contraction. In our study; 90 cases with dilated cardiomyopathy who were followed up between January 2005-December 2017 in Uludag University, Faculty of Medicine, Department of Pediatrics, Pediatric Cardiology Department, have been examined. Their age, gender distribution, symptoms at admission, etiologic factors, echocardiography, Holter and angiography results, medical and interventional therapies, treatment duration and response, healing, transplantation and mortality rates were evaluated. Of the 90 patients who formed the study group, 47,8% were male and 52,2% were female (male:43, female:47). The age of diagnosis was median: 11,5 months (minimum:0, maximum:234 months) and 58,9% of the cases were under the age of two. Mostly seen etiologic factors were cardiovascular diseases which observed 42,2% of patients (n:38) and idiopathic dilated cardiomyopathy which has seen 18,9% of patients (n:17). Patients of the 15,5% were asymptomatic. The most common symptom under the age of two was respiratory distress (60,4%) and in patients who are older than two years old were palpitation (27%) and also chest pain (10,8%). Totally 78,1% of the patients which were treated in the intensive care unit (ICU) were younger than two years of age and this rate was significantly higher than the group of patients who are older than two (21,9%), in need of ICU follow up (p<0,006). Of the patients with arrhythmia (n:11), 63.6% (n:7) recovered, 45,5% (n:5) of these patients were treated with medical therapy and 18,1% (n:2) of them recovered after ablation. The etiology of these patients who recovered by ablation, permanent reciprocating junction tachycardia (PJRT) and atrial ectopic tachycardia. Recovery did not occur in 1 patient who has Wolff-Parkinson-White Syndrome (WPW) even after ablation treatment. In 8 out of 22 patients whose arythtimias identified by Holter, aryhtmia was primary pathology, on the other hand the remaining 14 patients developed as a complication of DCMP. Angiography was performed in 44,4% (n:40) of the cases and abnormal left coronary artery originating from pulmoner artery (ALCAPA) was detected in 15% (n: 6) of these patients. Of the 18 patients with cardiovascular disease, had been performed balloon angioplasty or surgery, or with no procedure, 72,2% (n:13) recovered completely, 5,5% (n:1) continued their follow-up and 22,3% (n:4) of them were died. Follow-up period of patients was median:41 months (min:3 days, max:222,5 months). There was no difference in prognosis according to ages of patients significantly. An 18-year-old patient with thalassemia major who had a history of frequent blood transfusions underwent cardiac transplantation and recovered during follow-up. Consequently 37,7% of all patients recovered completely, 43,3% continued as they was, 12,2% were passed away. As a result; the data obtained from our study are important in terms of guiding the diagnosis, follow-up and treatment of dilated cardiomyopathy patients. Echocardiography is the most important diagnostic method in the follow-up of these patients, and with its widespread use, the recognition of etiologic factors such as left ventricular non-compaction CMP, other cardiovascular diseases that may cause DCMP and myocarditis has increased. Despite these advances in diagnosis and treatment, DCMP is still a disease with high morbidity and mortality. Key words: Dilated cardiomyopathy, echocardiography, left ventricular noncompaction, prognosis.