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Hypoplastic Left Heart Syndrome

By Jeanne Marie Baffa, MD, Associate Professor of Pediatrics;Program Director, Pediatric Cardiology Fellowship and Director of Echocardiography, Sidney Kimmel Medical College at Thomas Jefferson University;Nemours/Afred I. duPont Hospital for Children

Hypoplastic left heart syndrome is a birth defect in which the left side of the heart, including the lower heart chamber (left ventricle), heart valves (mitral and aortic valves), and aorta, does not develop sufficiently. Newborns with hypoplastic left heart syndrome also have an atrial septal defect (ASD) and a patent ductus arteriosus.

  • Hypoplastic left heart syndrome causes heart failure—the heart pumps less than normal amount of blood—and eventually death.

  • Diagnosis is by echocardiography.

  • Treatment is with a series of surgical procedures or with heart transplantation.

Hypoplastic is a medical term for underdeveloped. Hypoplastic left heart syndrome (HLHS) accounts for 2% of birth defects of the heart. Because the left side of the heart is underdeveloped, oxygenated blood coming into the left heart from the lungs is diverted into the right side of the heart through the atrial septal defect, where it mixes with deoxygenated blood returning to the heart from the body. This relatively deoxygenated blood exits the right heart to the lungs and through the ductus arteriosus to the body. The ductus arteriosus is a blood vessel connecting the two great arteries leaving the heart, the pulmonary artery and the aorta (see Normal Fetal Circulation). In the womb and during the first few days of life, the ductus is open. Blood flow to the body is maintained only because the ductus arteriosus remains open. Once the ductus closes, as it normally does after birth, very little blood flows to the body. If the defect is not repaired immediately, the infant will die.

Symptoms

Symptoms appear when the ductus arteriosus begins to close during the first 24 to 48 hours of life. Subsequently, signs of heart failure, including rapid breathing, shortness of breath, weak pulse, pale or bluish skin, cool body temperature, lethargy, and a decreased number of wet diapers, rapidly develop. When blood flow to the body is reduced, the heart, brain and other vital organs do not receive sufficient blood flow. Unless blood flow is re-established, the infant will die.

Diagnosis

  • Echocardiography

Many infants are diagnosed before birth when the birth defect is detected on a prenatal ultrasound examination done on the mother or on echocardiography done on the fetus.

Diagnosis is suspected when doctors notice symptoms during an examination of the infant. Diagnosis is confirmed by emergency echocardiography.

Chest x-rays and electrocardiography (ECG) may be done, and cardiac catheterization is sometimes needed.

Treatment

  • Drugs to keep the ductus arteriosus open until surgery

  • Surgical repair

  • Sometimes heart transplantation

Because most affected children with hypoplastic left heart syndrome are identified before birth, doctors begin giving a drug to keep the ductus open immediately after birth.

Immediate care

All affected infants are treated in a neonatal intensive care unit or pediatric cardiac intensive care unit. A prostaglandin is given by vein to prevent closure of the ductus arteriosus or to reopen a narrowed ductus. Newborns, particularly those that are critically ill, usually require assistance with breathing (mechanical ventilation). Severely ill newborns may require drugs to improve heart function.

Surgical repair

Survival ultimately requires a series of surgical procedures that enable the right ventricle to take over the work of the underdeveloped left ventricle.

Surgery is done is several stages:

  • Stage 1: Done during the first weeks of life

  • Stage 2: Done when the infant is 3 to 6 months of age

  • Stage 3: Done when the child is 18 to 36 months of age

Heart transplantation

In some infants, heart transplantation is considered the procedure of choice for hypoplastic left heart syndrome. Infants must be given an infusion of a prostaglandin to keep the ductus arteriosus open until a donor heart is available. Because availability of donor hearts is very limited, about 20% of infants die while awaiting transplant. The 5-year survival rates after transplantation and after multistage surgery are similar.

After heart transplantation, drugs to suppress the activity of the immune system (immunosuppressants) are required for the rest of the child's life. These drugs make people more susceptible to infections. Immunosuppressants may also damage the coronary arteries of the transplanted heart. The only known treatment for coronary artery damage is retransplantation.

Long-term care

Some children need to take antibiotics before visits to the dentist and before certain surgeries (such as on the bowel or bladder). These antibiotics are used to prevent serious heart infections called endocarditis.

Most children will take aspirin or a blood thinner such as warfarin or enoxaparin to help prevent clots from forming in the heart. Many affected children will need to take one or more other drugs to help their hearts continue to function optimally.

Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • LOVENOX
  • No US brand name
  • COUMADIN