Surgical Treatment for Double Outlet Right Ventricle With Pulmonary Outflow Tract Obstruction

World J Pediatr Congenit Heart Surg. 2016 Nov;7(6):696-699. doi: 10.1177/2150135116674440.

Abstract

Background: Double outlet right ventricle (DORV) is a conotruncal anomaly that is a defining element of many types of complex congenital heart disease. Because of a big variety of pathology, there are still some controversies with respect to the definition, classification, and surgical treatment. We report our experience with surgical treatment for DORV (as defined by the "90% rule") with pulmonary outflow tract obstruction (POTO).

Methods: From July 2005 to July 2015, 90 patients underwent surgical treatment of DORV with POTO at the First Hospital of Tsinghua University. There were 55 males and 35 females whose age varies from 3 months to 36 years (mean age 7.1 ± 9.0 years old), and body weights ranged from 5 to 63 kg (mean weight 20.4 ± 16.6 kg). Besides DORV, ventricular septal defect, and POTO, this group of patients includes some with additional associated cardiac abnormalities.

Results: Fourteen patients (15.6%) died. The main cause of death was low cardiac output syndrome.

Conclusions: The DORV is usually associated with a variety of cardiac abnormalities and POTO is a common defining feature. Acceptable surgical results can be achieved by individualized surgical treatment of most patients. Some patients may require reoperation, and a close follow-up is needed.

Keywords: cardiac abnormalities; double outlet right ventricle; pulmonary valve stenosis; surgery.

MeSH terms

  • Abnormalities, Multiple*
  • Adolescent
  • Adult
  • Cardiac Surgical Procedures / methods*
  • Child
  • Child, Preschool
  • Double Outlet Right Ventricle / diagnosis
  • Double Outlet Right Ventricle / surgery*
  • Female
  • Humans
  • Infant
  • Male
  • Pulmonary Valve Stenosis / congenital
  • Pulmonary Valve Stenosis / diagnosis
  • Pulmonary Valve Stenosis / surgery*
  • Treatment Outcome
  • Young Adult