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Doctor insights on: Oldest Living With Hypoplastic Left Heart

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What is hypoplastic left heart syndrome?

What is hypoplastic left heart syndrome?

A congenital defect: Hypoplastic left heart syndrome (hlhs) is a specific type of congenital heart defect that consists of varying degrees of underdevelopment of the left-sided heart structures including the mitral valve, left ventricle, aortic valve, and aortic arch. Hlhs most likely has a genetic cause and occurs early during fetal development, however the genes involved have not yet been entirely elucidated. ...Read more

Dr. John Charpie
34 doctors shared insights

Hypoplastic Left Heart (Definition)

This is a congenital heart defect that is characterized by the underdevelopment of the left side of the babies heart. The left ventricle is atrophic, the aortic and mitral valve are small and so is the ascending aorta and arch. These patients need surgical intervention. This is ...Read more


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What cuases hypoplastic left heart syndrome?

What cuases hypoplastic left heart syndrome?

Multifactorial.: 2-6% recurrence risk with 1-2 prior affected siblings and up to 25% risk with rare familial autosomal recessive forms - both point to genetic underpinnings. The pathological changes center around severe aortic and mitral valve stenoses and hypoplasia/interruption of the aortic arch. ...Read more

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Hypoplastic left heart syndrome, what to do?

Hypoplastic left heart syndrome, what to do?

Major defect: Limited options & generally fatal in 1st month w/o surgery. First must check for associated conditions: genetic, neurological, other conditions might preclude surgical intervention. Even in ideal candidate, options are generally transplant or staged palliation (series of 3 major surgeries in the first few years of life with a lifetime of care and potential procedures). It's a hard road. Good luck. ...Read more

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How is hypoplastic left heart syndrome cured?

How is hypoplastic left heart syndrome cured?

It cannot be cured: Hypoplastic left heart syndrome (hlhs) is a congenital defect that occurs early during fetal development and it cannot be cured; babies are born with it. It is possible to treat hlhs with surgical and/or catheter-based therapies, but the anatomic defects cannot be reversed. There are newer fetal interventions under investigation that may improve the outcomes, but they also are not curative. ...Read more

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Can you explain hypoplastic left heart syndrome?

Can you explain hypoplastic left heart syndrome?

Complex heart defect: 2-6% recurrence risk with 1-2 prior affected siblings and up to 25% risk with rare familial autosomal recessive forms - both point to genetic underpinnings for hlhs. The pathological changes center around severe aortic and mitral valve stenoses and hypoplasia/interruption of the aortic arch. ...Read more

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How is hypoplastic left heart syndrome diagnosed?

How is hypoplastic left heart syndrome diagnosed?

Ultrasound + cath: The DX is possible from a cardiac ultrasound & a cardiac cauterization. ...Read more

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What to do about hypoplastic left heart syndrome?

What to do about hypoplastic left heart syndrome?

Major defect: Limited options & generally fatal in 1st month w/o surgery. First must check for associated conditions: genetic, neurological, other conditions might preclude surgical intervention. Even in ideal candidate, options are generally transplant or staged palliation (series of 3 major surgeries in the first few years of life with a lifetime of care and potential procedures). It's a hard road. Good luck. ...Read more

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What are the symptoms of a hypoplastic left heart?

Do not survive: True hypoplastic left heart babies do not survive long enough to have symptoms (other than being cyanotic and very short of breath), they are usually diagnosed very quickly at birth and will have to have life-saving surgery within a few days of birth. ...Read more

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In what way is hypoplastic left heart syndrome treated?

In what way is hypoplastic left heart syndrome treated?

Surgery: Typically, a three surgery strategy is pursued. The first stage (norwood) is in the first few days of life. Stage 2 (glenn) is at 4-6 months, and stage 3 (fontan) is at age 2-4 years. The goals of this strategy are to provide reliable blood flow to the lungs and the body and to normalize the workload on the heart. Best results are in specialized congenital heart centers. ...Read more