Konjenital Olarak Düzelmiş Büyük Arter Transpozisyonlu Hastalarımızın Tanı Tedavi ve İzlem Süreçlerinin Değerlendirilmesi

Abstract

ABSTRACT MERAL A. Evaluation of diagnosis, treatment, and folllow-up process of our congenitally corrected transpositions of great arteries patients. Hacettepe University Faculty of Medicine, Department of Pediatrics Cardiology. Thesis for expertness in Pediatrics. Ankara, 2015. In this study, CCTGA patients diagnosed with echocardiography, catheterization and angiocardiography in the Department of Pediatric Cardiology at Hacettepe University School of Medicine were investigated retrospectively, and the course of their diagnosis, treatment and follow-up were evaluated. Data on patients diagnosed with CCTGA from 1985 to 2015 were compiled and analyzed. Additionally, patients with L-loop situs solitus, D-loop situs inversus, and atrial isomerism were also included in this study. The course of diagnosis, treatment, and follow-up for 57 patients, whose catheterization and echocardiography records located in the archieves, were evaluated. Of these 57 patients, 36 (%63,2) were male and 21 (%36,8) were female; and the male: female ratio was 1.7:1. At the time of diagnosis, the age range of patients was 0 days to 17 years, and the median age was 8 months. The most frequent symptom at presentation, seen in 18 (%31,6) patients, was cyanosis; while the most common physical examination finding, affecting 50 (%87,7) patients, was cardiac murmur. Cases with situs ambiguus and isolated levocardia were more commonly seen in our patient group compared to those reported in the literature. Among our patients, 37 (%64,9) had atrial septal defect (ASD) and 38 (%66,8) had ventricular septal defect (VSD), with perimembranous being the most common subtype (50%). Ebstein's anomaly was detected in the morphologic tricuspid valve of 7 (%12,3) patients. Obstruction of the morphologic left ventricle outflow tract (MLVOT) was seen in 31 (%54,4) patients. Among the ventriculoarterial connection anomalies, double-outlet morphologic right ventricle was the most common one (7%). Atrioventricular discordance and double-outlet morphologic left ventricle were seen together in only one patient. VSD with subaortic ridge was detected in 2 (%3,5) patients. Cardiac catheterization and angiography were performed for diagnostic purposes in 38 (%66,7) patients and interventional purposes in 10 (%17,6) patients. Among these 10 patients, 6 (%10,5) had cardiac pacemaker implantation, 3 (%5,3) had balloon v angioplasty, and 1 (%1,8) had conduit stent placement. The most common arrhythmia seen was complete atrioventricular block (16 (61,5%) patients). Among arrhythmia detected 26 patients, permenant pacemaker implantation was performed on 16 (61,5%) patients (Congenital complete AV block was seen in 5 (%19,2 patients; AV block was seen in 11 (%42,3) patients during and after surgery). Thirty one (%54,4) of the 57 patients studied went under 48 surgical procedures overall. None of the patients had complete anatomic repair or double-switch surgery. Among the 41 patients who had multiple visits for long term follow-up at our clinic, mean duration of follow-up was 107±85 days and the age range during follow-up was 2 months to 28 years. 6 patients died during follow-up, all of which were post-operative. Thirteen (%22,8) patients developed systemic ventricle dysfunction, and the mean age for emergence of the dysfunction was 7.04±5.6 (range: 0-17) years. In conclusion, the prognosis of c-TGA is associated with the accompanying cardiac defects. Cases with “situs ambiguus” and isolated levocardia were more commonly seen in our patient group compared to those reported in the literature. All of the interventions were conventional surgery. Mortality rate during childhood was low, except for surgical interventions. Key words: congenitally corrected transposition of great arteries, CCTGA, subaortic ridge, VSD, MLVOT, AV complete block, congenital heart disease