Hypoplastic left heart syndrome occurs during fetal development with symptoms developing days after birth.

Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. 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.

Causes

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.

Symptoms

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.

Why choose Ohio State for hypoplastic left heart syndrome treatment?

Anyone who has ever been diagnosed with 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

We offer 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.

Diagnosis

Some congenital heart defects can be detected before a baby is born. In other cases, severe heart defects are detected shortly after birth.

Tests used to diagnose hypoplastic left heart syndrome include:

Electrocardiogram (EKG/ECG) – A test that records the electrical activity of the heart

Echocardiogram (also called echo) – A test that uses sound waves to assess the function and structure of the heart muscle and valves

Chest X-ray – A radiograph or picture of the heart and lungs including blood vessels, ribs and bones of the spine

Cardiac MRI (cardiac magnetic resonance imaging or CMR) – A noninvasive, sophisticated imaging procedure that uses large magnets and a computer to produce detailed images of the structure and function of the heart while it is beating

Cardiac catheterization – A procedure to measure pressures in the heart and check for problems in the coronary arteries. A long, thin tube (catheter) is inserted into an artery or vein in the groin, arm or neck, and then threaded to the heart. The physician injects a contrast solution into the artery and takes X-rays to check for blockage and other abnormalities.

Treatments

Babies born with hypoplastic left heart syndrome must have a series of surgeries to treat the heart defect or a heart transplant. The most common treatment is a series of three operations that are performed to reconfigure the cardiovascular system to create normal blood flow in and out of the heart. These surgeries do not correct the defect but relieve the symptoms without resulting in a cure. Lifelong care is necessary after surgery.

Medications

If surgery is not immediately possible, medication can help to manage the condition. Prostaglandin, a medication that dilates the blood vessels, may be recommended before a baby is ready for surgery to keep the ductus arteriosus open and maintain adequate blood supply to the body.

Nonsurgical procedures

A cardiac catheterization procedure called atrial septostomy can enlarge the connection between the heart’s upper chambers and improve oxygen delivery throughout the body.

Surgical procedures

There are several surgeries that treat hypoplastic left heart syndrome and in some complex cases a heart transplant may be necessary.  

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