Research Article

Intensive cardiac management in patients with trisomy 13 or trisomy 18†

  1. Yukihiro Kaneko1,*,
  2. Jotaro Kobayashi1,
  3. Yusuke Yamamoto1,
  4. Hitoshi Yoda2,
  5. Yuki Kanetaka2,
  6. Yayohi Nakajima2,
  7. Daiichi Endo2,
  8. Keiji Tsuchiya3,
  9. Hajime Sato4,
  10. Tadashi Kawakami2

Article first published online: 15 APR 2008

DOI: 10.1002/ajmg.a.32311

Issue

American Journal of Medical Genetics Part A

American Journal of Medical Genetics Part A

Volume 146A, Issue 11, pages 1372–1380, 1 June 2008

Additional Information

How to Cite

Kaneko, Y., Kobayashi, J., Yamamoto, Y., Yoda, H., Kanetaka, Y., Nakajima, Y., Endo, D., Tsuchiya, K., Sato, H. and Kawakami, T. (2008), Intensive cardiac management in patients with trisomy 13 or trisomy 18. American Journal of Medical Genetics Part A, 146A: 1372–1380. doi: 10.1002/ajmg.a.32311

Author Information

  1. 1

    Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, Tokyo, Japan

  2. 2

    Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan

  3. 3

    Department of Pediatrics, Japanese Red Cross Medical Center, Tokyo, Japan

  4. 4

    Department of Public Health, Graduate School of Medicine, University of Tokyo, Tokyo, Japan

*Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935, Japan.

  1. †

    How to cite this article: Kaneko Y, Kobayashi J, Yamamoto Y, Yoda H, Kanetaka Y, Nakajima Y, Endo D, Tsuchiya K, Sato H, Kawakami T. 2008. Intensive cardiac management in patients with trisomy 13 or trisomy 18. Am J Med Genet Part A 146A:1372–1380.

Publication History

  1. Issue published online: 19 MAY 2008
  2. Article first published online: 15 APR 2008
  3. Manuscript Accepted: 24 JAN 2008
  4. Manuscript Received: 22 MAR 2007

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Keywords:

  • trisomy 13;
  • trisomy 18;
  • neonate;
  • congenital heart defects;
  • neonatal intensive care;
  • cardiac surgery

Abstract

Intensive cardiac management such as pharmacological intervention for ductal patency (indomethacin and/or mefenamic acid for closure and prostaglandin E1 for maintenance) and palliative or corrective surgery is a standard treatment for congenital heart defects. However, whether it would be a treatment option for children with trisomy 13 or trisomy 18 syndrome is controversial because the efficacy on survival in patients with these trisomies has not been evaluated. We retrospectively reviewed 31 consecutive neonates with trisomy 13 or trisomy 18 admitted to our neonatal ward within 6 hr of birth between 2000 and 2005. The institutional management policies differed during three distinct periods. In the first period, both pharmacological ductal intervention and cardiac surgery were withheld. In the second, pharmacological ductal intervention was offered as an option, but cardiac surgery was withheld. Both strategies were available during the third period. The median survival times of 13, 9, and 9 neonates from the first, second, and third periods were 7, 24, and 243 days, respectively. Univariate and multivariate analyses confirmed that the patients in the third period survived significantly longer than the others. Intensive cardiac management consisting of pharmacological intervention for ductal patency and cardiac surgery was demonstrated to improve survival in patients with trisomy 13 or trisomy 18 in this series. Therefore, we suggest that this approach is a treatment option for cardiac lesions associated with these trisomies. These data are helpful for clinicians and families to consider in the optimal treatment of patients with these trisomies. © 2008 Wiley-Liss, Inc.

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