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 1-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

Consistently ranked one of the nation's best cardiology and cardiac surgery programs by U.S. News and World Report.

  • With more than 40 pediatric cardiologists, we have experience diagnosing and treating every kind of heart problem.

    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. Our outcomes for the surgeries needed to reconfigure the heart’s circulation system are among the best in the nation.

    Seattle Children’s has been treating children since 1907. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.

  • Children with double inlet left ventricle receive compassionate, comprehensive care through our Single Ventricle Program.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.

  • Care often begins before a child is born, when their condition is diagnosed through our Prenatal Diagnosis and Treatment Program.

    We also have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready. This program was developed by Seattle Children’s and the University of Washington to help with care throughout your child’s life.

  • When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s cardiologist, you are connected with cardiac intensivists, neonatologists, nurses, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child’s health needs and help your family through this experience.

    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.

    Read more about the supportive care we offer.

Symptoms of Double Inlet Left Ventricle

Double inlet left ventricle allows oxygen-rich (red) and oxygen-poor (blue) blood to mix in the heart and get pumped out to your child’s body.

Your child may have symptoms like these: 

  • Getting tired easily
  • Bluish skin, lips or nailbeds (cyanosis) or pale skin
  • Trouble feeding
  • Trouble gaining weight and growing
  • Fast breathing or trouble breathing
  • Sweating more than normal
  • Swelling (edema) in the legs and abdomen

Diagnosing Double Inlet Left Ventricle

To diagnose this condition, your doctor will examine your child, check their heartbeat and listen to their heart. In children with double inlet left ventricle, doctors can often hear a heart murmur – the sound of blood moving in the heart in a way that’s not normal.

The doctor will ask for details about your child’s symptoms, their health history and your family health history.

Your child will also need tests that provide more information about how their heart looks and works. These tests may include: 

Treating Double Inlet Left Ventricle

Babies with double inlet left ventricle need surgery in the first weeks of life. They get a series of surgeries to redirect blood flow through their heart.

Double inlet left ventricle is referred to as a “single-ventricle heart defect” because there is only 1 pumping chamber in the heart (ventricle). Single-ventricle defects are some of the most complex heart-related birth defects.

We provide comprehensive care for children with double inlet left ventricle through our Single Ventricle Program.

  • Babies born with double inlet left ventricle usually have 3 surgeries during the first few years of life to reconfigure the circulation of the heart: 

    • The oxygen-poor blood, which returns from the body, is redirected to blood vessels that go to the lungs.
    • The oxygen-rich blood returns from the lung into the working ventricle. The working ventricle should be able to pump the oxygen-rich blood to the entire body.

     The exact procedures and timing of surgery depend on your child’s condition and any other heart defects.

  • Your child may need medicines like these before and after surgery: 

    • Angiotensin-converting enzyme (ACE) inhibitors, which lower blood pressure
    • Digoxin, which makes the heart beat slower and with more force
    • Anticoagulants, which help prevent or dissolve blood clots
  • In some cases, a heart transplant is needed. The heart transplant team at Seattle Children’s performs numerous transplants each year for children with this or other heart problems that cannot be controlled using other treatments. Read more about our Heart Transplant Program.

Follow-Up Care for Double Inlet Left Ventricle

Outcomes for children with double inlet left ventricle have improved dramatically in the past several decades. However, the period between the first and second surgeries represents an increased period of vulnerability for these infants. Children will be cared for by our Single Ventricle Program, which focuses on the care and monitoring of infants with these severe heart problems.

Seattle Children’s is part of a nationwide group of children’s hospitals called the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) dedicated to improving the outcomes and quality of life for children with heart defects. The NPC-QIC provides additional family resources, support and information.

Adult Congenital Heart Disease Program

To meet your child’s long-term healthcare needs, we have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready.

Contact Us 

Contact the Heart Center at 206-987-2515 to request an appointment, a second opinion or more information.

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