title: The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan.
abstract: Congenital heart defects (CHD) are very common in patients with trisomy 18 (T18) and trisomy 13 (T13). The surgical indication of CHD remains controversial since the natural history of these trisomies is documented to be poor. To investigate the outcome of CHD in patients with T18 and T13, we collected and evaluated clinical data from 134 patients with T18 and 27 patients with T13 through nationwide network of Japanese Society of Pediatric Cardiology and Cardiac Surgery. In patients with T18, 23 (17%) of 134 were alive at this survey. One hundred twenty-six (94%) of 134 patients had CHDs. The most common CHD was ventricular septal defect (VSD, 59%). Sixty-five (52%) of 126 patients with CHD developed pulmonary hypertension (PH). Thirty-two (25%) of 126 patients with CHD underwent cardiac surgery and 18 patients (56%) have survived beyond postoperative period. While palliative surgery was performed in most patients, six cases (19%) underwent intracardiac repair for VSD. Operated patients survived longer than those who did not have surgery (P < 0.01). In patients with T13, 5 (19%) of 27 patients were alive during study period. Twenty-three (85%) of 27 patients had CHD and 13 (57%) of 27 patients had PH. Atrial septal defect was the most common form of CHD (22%). Cardiac surgery was done in 6 (26%) of 23 patients. In this study, approximately a quarter of patients underwent surgery for CHD in both trisomies. Cardiac surgery may improve survival in selected patients with T18.
authors: Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
authors: Jun
authors: J
authors: Maeda
authors: Hiroyuki
authors: H
authors: Yamagishi
authors: Yoshiyuki
authors: Y
authors: Furutani
authors: Mitsuhiro
authors: M
authors: Kamisago
authors: Tadashi
authors: T
authors: Waragai
authors: Shinji
authors: S
authors: Oana
authors: Hiroki
authors: H
authors: Kajino
authors: Hiroyuki
authors: H
authors: Matsuura
authors: Katsuhiko
authors: K
authors: Mori
authors: Rumiko
authors: R
authors: Matsuoka
authors: Toshio
authors: T
authors: Nakanishi
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D025063:Chromosome Disorders / Q000453:epidemiology / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics*; D005260:Female; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000235:genetics* / Q000401:mortality / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000453:epidemiology / Q000235:genetics / Q000401:mortality / Q000601:surgery; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008423:Maternal Age; D010331:Paternal Age; D011795:Surveys and Questionnaires; D019616:Thoracic Surgical Procedures / Q000379:methods / Q000706:statistics & numerical data; D016896:Treatment Outcome; D014314:Trisomy / Q000235:genetics*; D000073839:Trisomy 13 Syndrome; D055815:Young Adult