NDLI: Intensive cardiac management in patients with trisomy 13 or trisomy 18.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Kaneko, Yukihiro Kobayashi, Jotaro Yamamoto, Yusuke Yoda, Hitoshi Kanetaka, Yuki Nakajima, Yayohi Endo, Daiichi Tsuchiya, Keiji Sato, Hajime Kawakami, Tadashi
Spatial Coverage	Japan
Description	Country affiliation: Japan Author Affiliation: Kaneko Y ( Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, Tokyo, Japan. yukihirokaneko@hotmail.com)
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.
File Format	HTM / HTML
ISSN	15524825
Issue Number	11
Volume Number	146A
e-ISSN	15524833
Journal	American Journal of Medical Genetics Part A
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2008-06-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Human Discipline Genetics Chromosomes, Human, Pair 13 Chromosomes, Human, Pair 18 Heart Defects, Congenital Complications Trisomy Cardiac Surgical Procedures Cohort Studies Drug Therapy Surgery Heart Failure Etiology Humans Infant, Newborn Intensive Care, Neonatal Japan Withholding Treatment Journal Article
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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