Hacettepe Üniversitesi Çocuk Kardiyoloji Bilim Dalı’nda 2000-2017 yılları arasında Ebstein anomalisi tanısı ile izlenilen hastaların değerlendirilmesi

Abstract

In this study, we aimed to evaluate and compare clinical characteristics, accompanying cardiac anomalies, treatment modalities (catheter, surgery, medical) administered to the patients, findings and results of follow-up of the patients diagnosed as Ebstein’s anomaly (EA) and determine the factors that may affect the disease prognosis. Data of the 79 EA patients followed at Hacettepe University Ihsan Dogramacı Children’s Hospital Pediatric Cardiology Department between years 2000 and 2017 were analysed within this study. Median age of the patients were 12.8 years (0.66-34.0). The most common accompanying cardiac anomaly was atrial septal defect, patent foramen ovale in 56.9%, followed by mild-moderate mitral regurgitation in 21.5% and ventricular septal defect in 16.5% of the patients. 75.9% of the patients had tricuspid regurgitation and severity of tricuspid regurgitation was mild in 11.7%, moderate in 38.3% and severe in 50.0% of the patients. 24 patients (30.3%) had arrhythmia; 20 of them (83.3%) had tachycardia and 4 of them (16.6%) had bradycardia. The most common arrhythmia was supraventricular tachycardia (SVT) and it was present in 79.1% (19/24) of the patients. Atrioventricular re-entrant tachycardia (AVRT) was observed in 15/19 (79.1%) of the arrhythmia patients and it was the most common SVT type. 15 patients had AVRT, 10 of them had Wolff-Parkinson-White syndrome, 1 of them had Mahaim tachycardia and 4 patients had no pre-excitation syndrome. 1 patient had atrioventricular nodal re-entrant tachycardia (AVNRT), 3 patients had intra-atrial re-entrant tachycardia (IART). 1 patient had a history of ventricular tachycardia. Atrioventricular (AV) block was detected in the 4 patients that had bradycardia; 2 of them had congenital complete AV block, 2 of them had postoperative complete AV block. Radiofrequency ablation (RFA) or cryoablation procedures were performed in 16/19 patients who had SVT. Total of 21 ablation procedures were performed in16 patients, 18 (85.7%) of them were RFA and 3 (14.3%) of them were cryoablation. 32.9% of the patients had surgical operation, 8.9% of the patients had second surgical operation. The type of surgical operation was biventricular repair in 11 patients, univentricular repair in 1 patient, one and half ventricle repair 1 patient and other surgical operations in 13 patients. Median follow-up of the patients were 2 years (0-25). Mortality rate was 10.3%. Statistically significant difference was detected with respect to age at diagnosis in between patients who had mortality and those who had not mortality (p=0.012). The patients who had mortality were younger at diagnosis than those who had no mortality. There was no statistically significant difference with respect to gender, clinical presentation, diagnosis during prenatal or postnatal period, accompanying arrhythmia between patients who had mortality and who had not (p>0.05).