Double Outlet Right Ventricle (DORV)

Double Outlet Right Ventricle (DORV) Affects the Heart’s Connection to Its Largest Artery, the Aorta.

Usually, the aorta is attached to the left side of the heart, and another artery called the pulmonary artery is attached to the right. This allows blood to flow through the lungs to receive oxygen, and then for that “oxygen-rich” blood to flow through the rest of the body.

But in babies with DORV, both arteries are attached to the right side of the heart, so blood can’t take the correct path. Blood that’s too low in oxygen gets pumped out to the body, while oxygen-rich blood flows back to the lungs. This puts a strain on the heart, which has to work harder than it should to make sure the body gets enough oxygen-rich blood.

Babies with DORV are also born with a ventricular septal defect (VSD), a hole in the wall between the lower left and lower right chambers of the heart. Their condition can be more or less serious depending the size and location of the VSD, and the exact position of the aorta and pulmonary artery.

Sometimes babies born with DORV have other congenital heart problems too, including:

  • Coarctation of the aorta
  • Mitral valve problems
  • Pulmonary atresia
  • Pulmonary valve stenosis
  • Right-sided aortic arch
  • Transposition of the great arteries (TGA)

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What Are the Signs and Symptoms of Double Outlet Right Ventricle?

DORV is often detected before birth, or in the first few days after a baby is born. Symptoms include:

  • Heart murmur
  • Bluish or grayish lips, skin and nails
  • Fast breathing
  • Sweating or clammy skin
  • Trouble feeding
  • Poor weight gain
  • Extreme sleepiness

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow. 
 

What Causes Double Outlet Right Ventricle?

DORV occurs before birth, when the heart doesn’t develop properly during early pregnancy. Doctors don’t know exactly what causes it.

Babies with certain genetic conditions — like trisomy 13, trisomy 18, or DiGeorge syndrome — are more at risk for DORV, but it also occurs in babies without any of these conditions.

How is Double Outlet Right Ventricle Treated?

Usually, babies with DORV need one or more heart surgeries during their first few years of life. The timing and type of these surgeries depend on the exact arrangement of their aorta and VSD, and any other heart problems or health issues.

Connecticut Children’s cardiac surgeons specialize in cutting-edge techniques to treat DORV. Our team is known for performing complex surgeries on even the tiniest babies, with outstanding surgical outcomes.

  • Intraventricular tunnel repair: Creates a tunnel from the VSD to the aorta and places a patch that controls the direction of blood flow
  • Arterial switch: Moves the aorta to the left side of the heart and closes the VSD
  • In rare cases, staged reconstruction known as “single ventricle palliation”: A series of open-heart surgeries to rebuild the heart and redirect how blood flows through it
  • Norwood procedure: Allows the right ventricle to pump blood to the body
  • Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
  • Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen

Most patients with a congenital heart defect, including DORV, need lifelong care from congenital heart experts. Connecticut Children’s Heart Center keeps patients connected to cardiologists who understand their unique anatomy, provides follow-up tests like echocardiograms at locations close to home, and continues congenital heart care in adulthood.