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Effectiveness of cardiac surgery in patients with trisomy 18: a single-institutional experience

Published online by Cambridge University Press:  28 January 2016

Yosuke Nakai ,
Miki Asano ,
Norikazu Nomura ,
Hidekazu Matsumae  and
Akira Mishima
Yosuke Nakai*
Affiliation:
Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Mizuho-ku, Nagoya, Japan
Miki Asano
Affiliation:
Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Mizuho-ku, Nagoya, Japan
Norikazu Nomura
Affiliation:
Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Mizuho-ku, Nagoya, Japan
Hidekazu Matsumae
Affiliation:
Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Mizuho-ku, Nagoya, Japan
Akira Mishima
Affiliation:
Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Mizuho-ku, Nagoya, Japan
*
Correspondence to: Y. Nakai, MD, Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences; 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan. Tel: +81 52 853 8099; Fax: +81 52 851 7201; E-mail: [email protected]
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Abstract

Background

Surgical repair for cardiac lesions has rarely been offered to patients with trisomy 18 because of their very short lifespans. We investigated the effectiveness of cardiac surgery in patients with trisomy 18.

Patients and methods

We performed a retrospective analysis of 20 consecutive patients with trisomy 18 and congenital cardiac anomalies who were evaluated between August, 2003 and July, 2013. All patients developed respiratory or cardiac failure due to excessive pulmonary blood flow. Patients were divided into two subgroups: one treated surgically (surgical group, n=10) and one treated without surgery (conservative group, n=10), primarily to compare the duration of survival between the groups.

Results

All the patients in the surgical group underwent cardiac surgery with pulmonary artery banding, including patent ductus arteriosus ligation in nine patients and coarctation repair in one. The duration of survival was significantly longer in the surgical group than in the conservative group (495.4±512.6 versus 93.1±76.2 days, respectively; p=0.03). A Cox proportional hazard model found cardiac surgery to be a significant predictor of survival time (risk ratio of 0.12, 95% confidence interval 0.016–0.63; p=0.01).

Conclusions

Cardiac surgery was effective in prolonging survival by managing high pulmonary blood flow; however, the indication for surgery should be carefully considered on a case-by-case basis, because the risk of sudden death remains even after surgery. Patients’ families should be provided with sufficient information to make decisions that will optimise the quality of life for both patients and their families.

Keywords

Trisomy 18cardiac surgerypulmonary artery bandingquality of life
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 7 , October 2016 , pp. 1391 - 1396
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Copyright
© Cambridge University Press 2016 

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