Heart and Blood Conditions

Double Inlet Left Ventricle

What is double inlet left ventricle?

Double inlet left ventricle is a rare birth defect in the heart.

Normally, the tricuspid valve leads into the right pumping chamber (ventricle), and the mitral valve leads into the left ventricle. These valves act as one-way doors, allowing blood to flow from the atria to the ventricles without letting blood back into the atria. A wall called the septum separates the ventricles.

In babies with this defect, both the tricuspid valve and the mitral valve let blood flow into the left ventricle. Only the left ventricle works. Because the right ventricle is not being used, it is small and not well developed.

This is also called a single-ventricle heart defect because children with this defect only have 1 ventricle in their heart that works. Single-ventricle defects are some of the most complex heart-related birth defects.

Double Inlet Left Ventricle at Seattle Children’s

Our heart team has treated many children with double inlet left ventricle. We have years of experience with the medical and surgical treatment these patients require. Children with double inlet left ventricle receive compassionate, comprehensive care through our Single Ventricle Program.

  • Care often begins before a child is born, when their condition is diagnosed through our Prenatal Diagnosis and Treatment Program.
  • Our outcomes for the surgeries needed to reconfigure the heart’s circulation system are among the best in the nation.
  • Through our Single Ventricle Interstage Clinic, we bring together experts in cardiology, nutrition, social work, feeding therapy and neurodevelopment to support your child’s health during the vulnerable transition between surgeries.
  • We also have a heart transplant program for children who need a transplant. Our team performs several transplants each year for children with double inlet left ventricle or other heart problems that cannot be controlled using other treatments.
  • We’re committed to your child’s overall health and well-being. We’ll discuss treatments in ways you can understand and involve you in every decision.
  • We have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready. This program was developed by Children’s and the University of Washington to help with care throughout your child’s life.

Double Inlet Left Ventricle in Children

This is a congenital defect. Doctors do not know why it happens. They think it occurs early in the pregnancy, when the baby’s heart is forming.

About 5 in every 100,000 babies have double inlet left ventricle.

Children with this defect may have other heart problems, too, like transposition of the great arteries, coarctation of the aorta, pulmonary atresia or pulmonary stenosis.

Contact Us

Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.