Outcomes of cardiac surgery in trisomy 18 patients

Cardiol Young. 2011 Apr;21(2):209-15. doi: 10.1017/S1047951110001848. Epub 2010 Dec 21.

Abstract

Objective: The objective was to clarify the outcomes of cardiac surgery in trisomy 18 patients.

Patients and methods: We analysed 34 consecutive trisomy 18 patients, of whom 21 were males, with cardiac complications. They were divided into patients who underwent cardiac surgery and those who were conservatively treated. We compared rates of survival and discharge alive between two groups.

Results: The surgery group included nine patients, with six males, who underwent cardiac surgery - intracardiac repair in three patients, pulmonary arterial banding in five patients, and ligation of the ductus in one patient - at median age of 2.2 months, ranging from 0.5 to 9.8, and with median weight of 2.6 kilograms, ranging from 1.5 to 3.2. Cardiac surgery and pre-operative assisted ventilation were hazardous factors leading to death. In the surgery group, cumulative survival rates at 1 month, 6 months, 12 months, and 24 months were 63%, 38%, 25%, and 22%, respectively, compared with 51%, 26%, 9%, and 9% in the conservative group. There was a significant difference (p = 0.002). The cumulative rates of discharge alive at 1 month, 3 months, and 6 months were 0%, 12%, and 65% in the surgery group, which did not differ from the conservative group (p = 0.80).

Conclusions: Cardiac surgery contributed to increased survival rate but not the rate of discharge alive in trisomy 18 patients. Cardiac surgery could not prevent all the trisomy 18 patients from death. The indication of cardiac surgery should be carefully individualised to improve the quality of life in trisomy 18 patients and concerned surrounding people.

Publication types

  • Comparative Study

MeSH terms

  • Cardiac Surgical Procedures*
  • Chromosomes, Human, Pair 18 / genetics
  • Female
  • Heart Defects, Congenital / genetics
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery*
  • Hospital Mortality / trends
  • Humans
  • Infant
  • Infant, Newborn
  • Japan / epidemiology
  • Male
  • Retrospective Studies
  • Survival Rate / trends
  • Treatment Outcome
  • Trisomy / genetics

Supplementary concepts

  • Chromosome 18, trisomy 18p