Diseases and Conditions

Double-outlet right ventricle

Overview Diagnosis Treatment

Treatment

Several types of surgery may be performed, depending on the specific heart defect and any associated defects.

Depending on the type of defect, surgeons may:

  • Create a tunnel through the ventricular septal defect to connect the left ventricle to the aorta
  • Switch the aorta and pulmonary artery positions, if they're reversed, in order to connect the pulmonary artery to the right ventricle and the aorta to the left ventricle
  • Insert a patch to close the hole between the ventricles
  • Insert a blood vessel to connect the right ventricle to the pulmonary artery, allowing more blood flow if the pulmonary artery is small
  • Widen a narrowed pulmonary artery to allow more blood flow
  • Conduct a series of other procedures to allow blood to move to the lungs and for the heart to function with one ventricle, if the defect is complex
  • Repair any other congenital heart defects

In some infants with inadequate blood flow to their lungs at birth, a temporary procedure may be done to insert a shunt between the aorta and the pulmonary artery. The shunt is removed later in life during heart surgery to repair the defect.

Adults who were born with double-outlet right ventricle will need lifelong care and regular follow-up exams. They should see a heart doctor trained in evaluating and treating congenital heart conditions (adult congenital cardiologist).

Surgery may be needed later in life for valve disease if there's a narrowing or leakage of the heart valves. Some adults need close monitoring of their aortas and pulmonary arteries, especially if they required surgery early in life. A small group of adults may require medications for treatment of decreased function involving the right or left ventricles.