Double outlet right ventricle

Double outlet right ventricle (DORV) is a condition in which both the pulmonary artery and the aorta connect to the right ventricle. DORVs are usually accompanied by one of a number of ventricular septal defects. The three types of VSDs most likely to occur with DORV are subaortic VSD (the defect is located beneath the aortic valve), subpulmonary VSD (the defect is located beneath the pulmonary valve) and doubly committed VSD (the defect is located within the septal band and immediately beneath the leaflets of the aortic and pulmonary valves). Sometimes the baby will have other associated cardiac defects.

Symptoms tend to occur early in life, often within days of birth. The two major symptoms are congestive heart failure and cyanosis. In some cases, cyanosis can become severe because of reduced pulmonary blood flow.

The goal of the surgical treatment of DORV is a complete anatomic repair. The left ventricle is connected to the aorta, the right ventricle is connected to the pulmonary artery and the VSD is closed. Usually a complete repair is undertaken at as early an age as possible.

Diagram 2.18 Double outlet right ventricle 1 - overriding aorta 2 - ventricular septal defect De-oxygenated blood enters the aorta from the right ventricle and is returned to the body.