title: Effectiveness of cardiac surgery in patients with trisomy 18: a single-institutional experience.
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.
authors.affiliation: Department of Cardiovascular Surgery,Nagoya City University Graduate School of Medical Sciences,Mizuho-ku,Nagoya,Japan.
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Nakai
authors.affiliation: Department of Cardiovascular Surgery,Nagoya City University Graduate School of Medical Sciences,Mizuho-ku,Nagoya,Japan.
authors.forename: Miki
authors.initials: M
authors.lastname: Asano
authors.affiliation: Department of Cardiovascular Surgery,Nagoya City University Graduate School of Medical Sciences,Mizuho-ku,Nagoya,Japan.
authors.forename: Norikazu
authors.initials: N
authors.lastname: Nomura
authors.affiliation: Department of Cardiovascular Surgery,Nagoya City University Graduate School of Medical Sciences,Mizuho-ku,Nagoya,Japan.
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Matsumae
authors.affiliation: Department of Cardiovascular Surgery,Nagoya City University Graduate School of Medical Sciences,Mizuho-ku,Nagoya,Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Mishima
mesh_terms: D006348:Cardiac Surgical Procedures; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000401:mortality* / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D011788:Quality of Life; D012189:Retrospective Studies; D016896:Treatment Outcome; D014314:Trisomy / Q000235:genetics*; D000073842:Trisomy 18 Syndrome; D014656:Vascular Surgical Procedures
keywords: Trisomy 18; cardiac surgery; pulmonary artery banding; quality of life