How to Cite this Article: Maeda J, Yamagishi H, Furutani Y, Kamisago M, Waragai T, Oana S, Kajino H, Matsuura H, Mori K, Matsuoka R, Nakanishi T. 2011. The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan. Am J Med Genet Part A 155: 2641–2646.
The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan†
Article first published online: 11 OCT 2011
Copyright © 2011 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part A
Volume 155, Issue 11, pages 2641–2646, November 2011
How to Cite
Maeda, J., Yamagishi, H., Furutani, Y., Kamisago, M., Waragai, T., Oana, S., Kajino, H., Matsuura, H., Mori, K., Matsuoka, R. and Nakanishi, T. (2011), The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan. Am. J. Med. Genet., 155: 2641–2646. doi: 10.1002/ajmg.a.34285
- Issue published online: 20 OCT 2011
- Article first published online: 11 OCT 2011
- Manuscript Accepted: 29 JUL 2011
- Manuscript Received: 9 DEC 2010
- trisomy 18;
- trisomy 13;
- cardiac surgery
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. © 2011 Wiley Periodicals, Inc.