Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. Babies who have this condition may seem normal at birth, but the condition quickly becomes noticeable within a few days of life as the opening between the right and left side of the heart begins to close. The baby then becomes ashen, has difficulty breathing, and cannot eat because the heart is too small to supply blood, oxygen and nutrients to the body.
Treatment includes a series of complex surgeries or a heart transplant. Infants who are not treated within the first days of life often die.
Why Choose The Ohio State University Wexner Medical Center?
Anyone who has ever been diagnosed with and/or treated for hypoplastic left heart syndrome should have lifelong care from a cardiologist who specializes in congenital heart defects. The Ohio State University Wexner Medical Center provides this care from birth to adulthood. We partner with Nationwide Children’s Hospital to provide the resources necessary for the care of adult congenital cardiac patients through the Columbus Ohio Adult Congenital Heart Disease Program (COACH).
The COACH program focuses on:
- Adults with congenital heart disease (CHD)
- Pulmonary hypertension
- Cardiovascular connective tissue disorders
- Pregnancy in women with heart disease
- Transition of adolescents with CHD into adult congenital heart disease (ACHD) care
- Training of future ACHD providers
Ohio State’s Wexner Medical Center offers a team approach to CHD, which means each patient is evaluated by an Adult Congenital Heart Disease (ACHD) specialist, and when appropriate, a cardiac surgeon, an imaging specialist and an interventional cardiologist with training in CHD. This integrated approach means that each patient’s disease is treated individually, with that particular patient’s needs, and physical condition, in mind.
What Is Hypoplastic Left Heart Syndrome?
The underdeveloped left side of the heart in hypoplastic left heart syndrome results in an inability to properly pump blood to the body. Immediately after birth, and for a few days, the right side of the heart compensates, pumping blood to the lungs and to the rest of the body. After a few days, the opening (patent ductus arteriosus) between the two sides of the heart closes, meaning that the right side of the heart can no longer pump blood out to the body.
Many babies who have hypoplastic left heart syndrome also have atrial septal defect, or a hole in the wall between the upper chambers of the heart. This defect allows the right side of the heart to pump blood to the body after the natural connection closes. If the baby does not have atrial septal defect or the natural connection closes, the baby can go into shock or die.
What Causes Hypoplastic Left Heart Syndrome?
Hypoplastic left heart syndrome occurs during fetal development. The cause is unknown although genetics may play a role. If your family has a history of congenital heart defects, you may want to consider consulting with a genetic counselor, who may recommend genetic testing.