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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

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How is hypoplastic left heart syndrome usually ?Treated?

How is hypoplastic left heart syndrome usually ?Treated?

Norwood/Glenn/Fontan: This sequence of major cardiac surgeries can help manage this severe congenital cardiac anomaly of the left heart. Significant intra- and inter-procedural mortality remains and the option of heart transplantation may be on the table. See: http://texaschildrens.Org/locate/doctors/ayres, -nancy/. ...Read more

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Could you have a child with hypoplastic left heart syndrome?

Could you have a child with hypoplastic left heart syndrome?

Yes: Although a rare defect overall, hlhs is a relatively common congenital heart defect. While hlhs is a very serious, life-threatening heart problem, outcomes continue to improve, particularly in experienced congenital heart centers. ...Read more

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Are there different?Types of hypoplastic left heart syndrome?

Are there different?Types of hypoplastic left heart syndrome?

Yes.: Permutations of the fetal syndrome have profound impact on prognosis - specifically whether the interatrial septum is widely patent or restrictive. http://texaschildrens.org/locate/doctors/ayres, -nancy/. ...Read more

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What age can some 1 live 2 with hypoplastic left heart syndrome?

What age can some 1 live 2 with hypoplastic left heart syndrome?

Tough to say: I've seen patients live into their 20's with hlhs, but it is hard to imagine how different things will be 20 years from now. An excellent surgeon is a crucial factor. ...Read more

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How does anyone know someone has hypoplastic left heart syndrome?

How does anyone know someone has hypoplastic left heart syndrome?

Echocardiogram: An infant will be suspected as having heart defect by examination and pulse oximetry. A pediatric cardiologist will perform an echocardiogram to make the diagnosis. The infant may appear very normal on the outside. Once changes occur in the newborn infant hlhs is not compatible with life without surgical intervention. A fetal echo by an experienced ped cardiologist can id this before birth. ...Read more

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What is the definition or description of: Hypoplastic left heart?

What is the definition or description of: Hypoplastic left heart?

Small left heart: 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 a life threatening condition. ...Read more

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Does anyone know someone that has hypoplastic left heart syndrome?

Does anyone know someone that has hypoplastic left heart syndrome?

Yes, lots of kids: I'm a pediatric cardiologist and caediac intensive care physician at one of the largest congenital heart programs in the country, so i've taken care of many children with hypoplastic left heart syndrome. ...Read more

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Anybody know somebody that survived hypoplastic left heart syndrome?

Yes: As a pediatric cardiologist, I have many patients who have hypoplastic left heart syndrome. Many are doing rather well, attending school. Obviously all do not survive, and some struggle with serious health issues and multiple medications. The "long" long term is not known at this time. Just over 20 years ago many doctors offered no surgery, and just hospice type care. We've come a long way. ...Read more

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Is it possible?For a baby to survive with ?Hypoplastic left heart syndrome?

Is it possible?For a baby to survive with ?Hypoplastic left heart syndrome?

Sure!: The perinatal mortality and infant mortality with hlhs is at least 30%, even in the best centers of congenital heart defects. It is uniformly lethal if undiagnosed/untreated. See: http://texaschildrens.Org/locate/doctors/ayres, -nancy/. ...Read more

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Is it possible to have more than 1 child with hypoplastic left heart syndrome?

Is it possible to have more than 1 child with hypoplastic left heart syndrome?

Genetic components: Hypoplastic left heart syndrome, in some cases, appears to have a familial (genetic) component. There are various genes that have been suggested to have influence over cardiac development where mutations in one of those genes may result in hlhs. Additionally, there are families where bicuspid aortic valve and hlhs (and other left-sided obstructive lesions) have related inheritance. ...Read more