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Pediatric Hypoplastic Left Heart Syndrome (HLHS)

Hypoplastic left heart syndrome (HLHS) is a combination of several abnormalities of the heart and great blood vessels. It is a congenital (present at birth) syndrome, meaning that the heart defects occur due to underdevelopment of sections of the fetal heart beginning during the first eight weeks of pregnancy. 

In the normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle and then is pumped out to the body through the aorta.

In hypoplastic left heart syndrome, most of the structures on the left side of the heart are small and underdeveloped. The degree of underdevelopment differs from child to child. The structures affected usually include the following:

  • Mitral valve. The valve that controls blood flow between the left atrium and left ventricle in the heart.

  • Left ventricle. The lower left-hand chamber of the heart. It receives oxygen-rich (red) blood from the left atrium and pumps it into the aorta, which takes the blood to the body. The left ventricle must be strong and muscular in order to pump enough blood to the body to meet its requirements.

  • Aortic valve. The valve that regulates blood flow from the heart into the aorta.

  • Aorta. The largest artery in the body and the primary blood vessel leading from the heart to the body.

Anatomy of a heart with hypoplastic left heart syndrome

Perhaps the most critical defect in HLHS is the small, underdeveloped left ventricle. This chamber is normally very strong and muscular so it can pump blood to the body. When the chamber is small and poorly developed, it will not function effectively and cannot provide enough blood flow to meet the body's needs. For this reason, an infant with hypoplastic left heart syndrome will not live long without surgical intervention:

  • Hypoplastic left heart syndrome occurs in 2 to 3 out of every 10,000 live births or 1 in every 4,344 babies born in the U.S. each year. 

  • HLHS occurs slightly more often in boys than in girls.

View information about our surgical volumes and outcomes at Children's National Hospital.

Treatments

Treatments

Treatment Services

Heart Surgery

Our pediatric heart specialists have dedicated their careers to providing excellent heart care to patients with congenital heart defects and other cardiac conditions. Learn more about pediatric heart surgery.

Children's Team

Children's Team

Providers

Yves d'Udekem

Yves d'Udekem

Division Chief, Cardiac Surgery
Co-Director, Children's National Heart Institute
 Mary Donofrio

Mary Donofrio

Medical Director, Prenatal Cardiology Program
Co-Director, Cardiac Neurodevelopmental Outcome Program
Advanced Imaging Cardiologist
Richard Jonas

Richard Jonas

Emeritus Chief, Cardiac Surgery
Senior Investigator, Center for Neuroscience Research
Can Yerebakan

Can Yerebakan

Associate Chief, Cardiac Surgery
Director, HLHS Hybrid Program
Director, Cardiovascular Surgery Fellowship Program
Locations that Treat this Condition

Locations that Treat this Condition

Show more Locations
Our Stories

Our Stories

Ashely smiling

Ashley's Story

Between her premature birth and her medical issues including her critical HLHS heart condition, her parents, Ana Mercedes and Axel Vela, were told that Ashley was unlikely to survive.
Cayden and Reagen as babies laying next to eachother sleeping

Cayden's Story

Cayden was born with a life-threatening complex congenital heart defect. Learn how the hybrid procedure for HLHS helped Cayden.

Departments

Departments

Cardiac Surgery

Our pediatric heart surgery team performs twice the number of surgeries of any other hospital in the region, with some of the best outcomes in the nation.

Children's National Heart Institute

Our expert pediatric heart team, including more than 40 subspecialties, offer advanced heart care and excellent outcomes for thousands of children every year.