The evolving treatment of congenital heart disease in patient with Down syndrome: Current state of knowledge

J Card Surg. 2022 Nov;37(11):3760-3768. doi: 10.1111/jocs.16875. Epub 2022 Aug 21.


Objectives: Children with Down syndrome are usually seen as not worthy of high-risk cardiac surgery. Through this review, we try to show the results of curative and palliative surgery for functional single ventricle syndrome in patients with Down syndrome, as there is currently no standard protocol for the treatment of this category of patients.

Methods: An exhaustive search of all related published medical literature included the following domains: Down syndrome and diagnosis, Down syndrome and taxonomy, Down syndrome, and natural history, Down syndrome and cardiovascular abnormalities, Down syndrome and pulmonary hypertension, Down syndrome and institutionalization, Down syndrome and surgical repair, Down syndrome, and single ventricle palliation, Down syndrome and Glenn, Down syndrome, and Fontan.

Results: 12 articles were included from 775 identified. Low-risk cardiac surgery procedure should be provided for Down syndrome with a balanced ventricular septal defect. There is no universal agreement about the surgical approach for Down syndrome with unbalanced ventricular septal defects, but it can be performed at relatively low risk.

Conclusions: TCPC in Down syndrome patients could be a relatively low-risk procedure if patients are prepared well and their pulmonary vascular resistance is low. Randomized prospective studies are required to show the long-term impact of TCPC palliation and develop a better understanding of standardized care of these patients.

Keywords: Down syndrome; balanced AVSD; congenital heart disease; single ventricle palliation; unbalanced AVSD.

Publication types

  • Review

MeSH terms

  • Child
  • Down Syndrome* / complications
  • Fontan Procedure* / methods
  • Heart Defects, Congenital* / complications
  • Heart Defects, Congenital* / diagnosis
  • Heart Defects, Congenital* / surgery
  • Heart Ventricles / surgery
  • Humans
  • Palliative Care / methods
  • Retrospective Studies
  • Treatment Outcome