13번, 18번 삼염색체 증후군 환자에서 선천성 심장병 수술이 적절한 치료법인지?

13번, 18번 삼염색체 증후군 환자에서 선천성 심장병 수술이 적절한 치료법인지?

Is cardiac surgery for trisomy 13 or 18 valuable option? - unsolved clinical and ethnic issue

(구연):
Release Date : 2017. 10. 27(금)
Se Yong Jung, Jung Yoon Kim, Ah Young Kim , Jae Young Choi , Jo Won Jung
Yonsei Univeresity College of Medicine Division of Pediatric Cardiolgoy1
정세용, 김정윤, 김아영, 최재영 , 정조원
연세대학교 의과대학 소아과학교실 소아심장과 1

Abstract

The clinical and ethnic decision of surgery for congenital heart disease (CHD) for trisomy 13 (T13) or trisomy 18 (T18) remains controversial. However, recent data showed that corrective cardiac surgery for simple CHD or palliative cardiac surgery for complex lesion may have short-term survival benefit, although long-term survivla benefit or improvement for delayed development is uncertain. We retrospectively reviewed clinical data of T13 or T18 patients diagnosed during recent 10 years. Variants of T13 or T18 were excluded due to distinct nature, and total of 14 patients (8 with T13 and 6 with T18) were reviewed. Mean follow-up duration was 5 months (range, 0.1 to 22.3). Half of patients were died within 3 months, and 2 (14.2%) patients survived for 1 years, and 4 patients (28.4%) were still alive. For cardiac problem, double oulet right ventricle (n=5, tetralogy of fallot type, n=3 or with endocardial cushion defect, n=2) were most common followed by ventricular setpal defect (n=4), atrial septa defect (n=2), pulmonary atresia with intact ventricular septum (n=1), coarctation of aorta (n=1) and hypoplastic left heart syndrome (n=1). Neurologic problem was occured in most patients (13/14, 92.8%), and most common problem was agenesis of corpus callosum. Cardiac surgery was perfomed in 5 patients (corrective surgery and one palliative). Cardiac surgery group have longer survival (9.8 vs 2.2 months, p = 0.022), although there was one mortality related with postoperative arrhythmia. Except early mortality, 4 patients survived for 8.9 month (range 5.0 to 14.5). Individual clinical and ethnic assesment for CHD surgery is mandatory for these patients.

Keywords: Edward syndrome, Patau syndrome, cardiac surgery