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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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Hypoplastic Left Heart Syndrome (Developments in Cardiovascular Medicine) $117.60 Hypoplastic Left Heart Syndrome: A Handbook for Parents $180.00
Hypoplastic Left Heart Syndrome $43.99 Ethical principles and parental choice: treatment options for neonates with hypoplastic left heart syndrome. (Pediatric Ethics, Issues, & Commentary). : An article from: Pediatric Nursing $5.95
Hypoplastic Left Heart Syndrome $100.35 Hypoplastic left heart syndrome. (Letters to the Editor).(Brief Article) : An article from: Pediatric Nursing $5.95
Hypoplastic Left Heart Syndrome. : An article from: Pediatric Nursing $5.95 Hypoplastic Left Heart Syndrome: A Medical Dictionary, Bibliography, And Annotated Research Guide To Internet References $25.44

Hypoplastic left-heart syndrome : the first description of the pathophysiology in 1851; translation of a publication by Dr. Bardeleben from Giessen, Germany
(CHEST 2001; 120.1368-1371) Key words: earliest illustration; historical article; hypoplastic left heart syndrome The term hypoplastic left-heart ...
Hypoplastic left heart syndrome associated with congenital right-sided diaphragmatic hernia and omphalocele - Selected Reports
Hypoplastic left heart syndrome (HLHS) is a prevalent congenital cardiac anomaly with high mortality.[1-3] The focus of perioperative management is on maintaining adequate pulmonary-to-systemic cir
Management of infants with hypoplastic left heart syndrome integrating research into nursing practice
Congenital heart disease resulting in defects of the heart and circulatory system affects more infants than any other type of birth defect and occurs ...
Congenital heart disease
Congenital heart disease includes a variety of malformations of the heart or its major blood vessels that are present at the birth of a child.
Pediatric heart transplantation across ABO blood type barriers: a case study
Heart transplantation with ABO blood type-incompatible donors has historically been contraindicated because of the high risk of an immediate hyperacute ...
Prenatal diagnosis of heart defects aids survival after surgery - News - Brief Article
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 ...
Surgical treatment in neonates with aortic arch obstruction or interruption with functional single ventricle
PURPOSE: The neonate with a functional single ventricle (FSV) and aortic arch obstruction or interruption other than hypoplastic left heart syndrome ...
Preoperative and Postoperative Endotoxemia in Children With Congenital Heart Disease - )
Study objectives: Recent data indicate that increases in inflammatory cytokines are seen in patients with diverse cardiac diseases. However, the primary ...

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