Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy

J Thorac Cardiovasc Surg. 2011 Mar;141(3):637-44, 644.e1-3. doi: 10.1016/j.jtcvs.2010.07.082. Epub 2010 Sep 29.


Objective: Patients with heterotaxy and complex congenital heart disease underwent cardiac surgery with high mortality and morbidity. Recent studies have revealed an association among heterotaxy, congenital heart disease, and primary ciliary dyskinesia. We undertook a retrospective review of patients undergoing cardiac surgery at Children's National Medical Center between 2004 and 2008 to explore the hypothesis that there is increased mortality and respiratory complications in heterotaxy patients.

Methods: Retrospective review was performed on postsurgical outcomes of 87 patients with heterotaxy and congenital heart disease exhibiting the full spectrum of situs abnormalities associated with heterotaxy. As controls patients, 634 cardiac surgical patients with congenital heart disease, but without laterality defects, were selected, and surgical complexities were similar with a median Risk Adjustment in Congenital Heart Surgery-1 score of 3.0 for both groups.

Results: We found the mean length of postoperative hospital stay (17 vs 11 days) and mechanical ventilation (11 vs 4 days) were significantly increased in the heterotaxy patients. Also elevated were rates of tracheostomies (6.9% vs 1.6%; odds ratio, 4.6), extracorporeal membrane oxygenation support (12.6% vs 4.9%: odds ratio, 2.8), prolonged ventilatory courses (23% vs 12.3%; odds ratio, 2.1) and postsurgical deaths (16.1% vs 4.7%; odds ratio, 3.9).

Conclusions: Our findings show heterotaxy patients had more postsurgical events with increased postsurgical mortality and risk for respiratory complications as compared to control patients with similar Risk Adjustment in Congenital Heart Surgery-1 surgical complexity scores. We speculate that increased respiratory complications maybe due to ciliary dysfunction. Further studies are needed to explore the basis for the increased surgical risks for heterotaxy patients undergoing cardiac surgery.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple*
  • Adolescent
  • Adult
  • Cardiac Surgical Procedures / adverse effects*
  • Cardiac Surgical Procedures / mortality
  • Case-Control Studies
  • Chi-Square Distribution
  • Child
  • Child, Preschool
  • Dextrocardia / complications
  • Dextrocardia / mortality
  • District of Columbia
  • Extracorporeal Membrane Oxygenation
  • Female
  • Genetic Diseases, X-Linked / complications
  • Genetic Diseases, X-Linked / mortality
  • Heart Defects, Congenital / complications
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery*
  • Heterotaxy Syndrome
  • Humans
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Kartagener Syndrome / complications
  • Kartagener Syndrome / mortality
  • Length of Stay
  • Male
  • Odds Ratio
  • Respiration Disorders / etiology*
  • Respiration Disorders / mortality
  • Respiration Disorders / therapy
  • Respiration, Artificial
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Situs Inversus / complications
  • Situs Inversus / mortality
  • Tracheostomy
  • Treatment Outcome
  • Young Adult

Supplementary concepts

  • Heterotaxy, visceral, X-linked