Cardiac surgery for children with trisomies 13 and 18: Where are we now?

Semin Perinatol. 2016 Jun;40(4):254-60. doi: 10.1053/j.semperi.2015.12.015. Epub 2016 Feb 1.


The objective is to examine whether cardiac surgery should be considered for children with trisomy 13 or 18 (T13 or 18).T13 or 18 were previously referred to as "lethal" conditions due to high mortality rates and severe disability among survivors. In the last decade, investigations have revealed these conditions are heterogeneous, with increasing numbers of studies describing interventions for these children. A number of factors makes the interpretation of reported outcomes after cardiac surgery challenging: (1) dissimilarities in practice lead to a wide variation in reported outcomes after cardiac surgery; (2) cardiac surgery is generally offered to older, healthier children; (3) cardiac surgeries of widely varying risks are often lumped together in individual studies, and (4) cases where cardiac surgery has been withheld are generally not included in publications. It is unclear whether withholding cardiac surgery for some children with a ventricular septal defect will lead to death, or the development of pulmonary hypertension, or if death will occur from other causes. In this article, we describe two children with different clinical situations and examine whether cardiac surgery would benefit them and how to communicate with their families. Cardiac surgery may be beneficial to some children with trisomy 13 or 18, but may harm others. Every child should be approached in an individual fashion and the goals of each family should be addressed. Children who are more likely to benefit from surgery may be older, healthier children without respiratory support. Rigorous and transparent research is needed to identify factors that affect survival in trisomy 13 or 18.

Keywords: Cardiac surgery; Clinical ethics; Communication; Congenital anomalies; Decision-making; Empathy; End of life decisions; Ethics; Family centered care; Life-sustaining interventions; Palliative care; Trisomy 13; Trisomy 18.

Publication types

  • Review

MeSH terms

  • Cardiac Surgical Procedures* / ethics
  • Cardiac Surgical Procedures* / mortality
  • Chromosomes, Human, Pair 13
  • Chromosomes, Human, Pair 18
  • Family Nursing / ethics
  • Female
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery*
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Precision Medicine / ethics*
  • Quality of Life
  • Risk Factors
  • Terminal Care* / ethics
  • Trisomy 13 Syndrome / mortality
  • Trisomy 13 Syndrome / surgery*
  • Trisomy 18 Syndrome / mortality
  • Trisomy 18 Syndrome / surgery*
  • Value of Life