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Hypoplastic left heart syndrome

In hypoplastic left heart syndrome, the left side of the heart - including the aorta, aortic valve, left ventricle and mitral valve - is underdeveloped. Blood returning from the lungs must flow through an opening in the wall between the atria (atrial septal defect). The right ventricle pumps the blood into the pulmonary artery and blood reaches the aorta through a patent ductus arteriosus. more...

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The baby often seems normal at birth, but will come to medical attention within a few days of birth as the ductus closes. Babies with this syndrome become ashen, have rapid and difficult breathing and have difficulty feeding. This heart defect is almost always fatal within the first days or months or life unless it's treated.

Although this defect is not correctable, some babies can be treated with a series of operations, or a heart transplantation. Until an operation is performed, the ductus is kept open by an intravenous medication containing prostaglandin. Because these operations are complex and need to be individualized for each patient, it's necessary to discuss all the medical and surgical options.

The surgery is done in several stages. The first stage, referred to as the Norwood procedure, allows the right ventricle to pump blood to both the lungs and the body. It must be performed soon after birth. The final stage(s) has many names, including bi-directional Glenn, Fontan operation, and lateral tunnel. These operations create a connection between the veins returning blue blood to the heart and the pulmonary artery. The overall goal of the operation is to allow the right ventricle to pump only oxygenated blood to the body and to prevent or reduce mixing of the red and blue blood. Some infants require several intermediate operations to achieve the final goal.

Some doctors will recommend heart transplantation to treat this problem. Although it does provide the infant with a heart that has normal structure, the infant will require life-long medications to prevent rejection. Many other transplant-related problems can develop, and these should be discussed with your doctor.

Children with hypoplastic left heart syndrome require lifelong follow-up by a cardiologist for repeated checks of how their heart is working. Virtually all the children will require heart medicines. They also risk infection on the heart's valves (endocarditis) and will need antibiotics such as amoxicillin before dental work and certain surgeries to help prevent endocarditis. Good dental hygiene also lowers the risk of endocarditis. For more information about dental hygiene and preventing endocarditis, ask your pediatric cardiologist.

External Links

  • Hypoplastic Left Heart Syndrome information from Seattle Children's Hospital Heart Center

Sources

  • Hypoplastic Left Heart Syndrome, American Heart Association
  • Card-AG, The Cardiologycal Working Group of the University Pediatric Clinic Munster

Read more at Wikipedia.org


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Prenatal diagnosis of heart defects aids survival after surgery - News - Brief Article
From British Medical Journal, 3/17/01 by David Spurgeon

Babies in whom a heart defect was diagnosed before birth were more likely to survive initial surgery to correct the defect than those in whom a defect was diagnosed after birth, a new study has shown. Only two thirds of those with a postnatal diagnosis survived.

Dr Wayne Tworetzky, lead investigator of the study, said: "This is the first study to demonstrate that patients diagnosed prenatally with hypoplastic left heart syndrome have improved survival after the first stage of surgery compared to those diagnosed after birth" (Circulation 2001;103:1269-73).

Dr Tworetzky is a clinical instructor in the cardiology department at Boston Children's Hospital. The study, which used standard prenatal ultrasound, was carried out at the University of California, San Francisco.

Previous studies did not show a significant benefit, but Dr Tworetzky said that these were smaller and may not reflect recent medical advances in managing such cases. This study dealt with 88 infants in whom the condition was diagnosed at the university between July 1992 and March 1999: it was diagnosed prenatally in 33 cases and postnatally in 55. Only 52 of the babies underwent surgery, and the survival rate among those with a prenatal diagnosis was higher than among the rest.

In the heart condition studied, the left side of the heart is underdeveloped and unable to support blood circulation after birth. Without treatment, babies with this defect usually die within the first few days of life. Treatment entails a series of operations.

Dr Tworetzky said that prenatal diagnosis affords time for physicians both to counsel parents and to optimise early treatment, which allows the infant to be in the best condition for surgery. Without the benefit of prenatal diagnosis, many babies with this condition are born in a facility that is not equipped to care for them.

He said: "The delay in diagnosis may lead to the infant going into shock or suffering multiple organ damage among other complications--all of which limit the success of the initial surgery ... The normal changes that occur in the newborn infant's circulatory system shortly after birth are not well tolerated by these infants. The first operation is the most difficult and carries the highest risk of death."

Overall survival from surgery to hospital discharge was 75%, but all of the babies with a prenatal diagnosis survived to discharge. Those with a postnatal diagnosis were generally more ill before going into surgery and had poorer outcomes after surgery: about 66% survived the surgery.

Among the 52 infants who underwent surgery, those with a prenatal diagnosis also had a significantly lower incidence of preoperative lactic acidosis. These babies also had lower rates of ventricular dysfunction and were significantly less likely to need drugs for resuscitation before the operation.

Although hypoplastic left heart syndrome is one of the most common cardiac defects found prenatally, Dr Tworetzky said that it is still common for the condition to be diagnosed after birth. The mothers of most babies with a postnatal diagnosis in this study had not undergone routine prenatal ultrasound screening. There are no known risk factors or genetic causes for the condition, but research is under way to fred possible genetic links.

David Spurgeon Quebec

COPYRIGHT 2001 British Medical Association
COPYRIGHT 2001 Gale Group

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