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Doctor insights on: Hypoplastic Left Heart In Children

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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

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Anybody have or know somebody that survived who has hypoplastic left heart syndrome?

Anybody have or know somebody that survived who has hypoplastic left heart syndrome?

Absolutely: In fact, in the current era, in experienced centers, survival for hypoplastic left heart syndrome exceeds 80%. Hypoplastic left heart syndrome, unfortunately, is fatal without surgery at a very early age, though. ...Read more

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What are the chances of having more then one child with hypoplastic left heart syndrome?

What are the chances of having more then one child with hypoplastic left heart syndrome?

Tiny: The genetic influences on heart formation/defects are complex and located on several separate chromosomes. The risk of you having another kid with ANY heart defect (there are lots of them) is about 4%, while that of the same defect is incredibly small. ...Read more

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I am having more then one child with hypoplastic left heart syndrome, what could cause this?

I am having more then one child with hypoplastic left heart syndrome, what could cause this?

Can be familial: 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

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My son has hypoplastic left heart syndrome and he is 11 months. He hasn't been circumsized. So how do I ho about to get him circumsized?

My son has hypoplastic left heart syndrome and he is 11 months. He hasn't been circumsized. So how do I ho about to get him circumsized?

When at the hospital: If your son has hlhs and is 11 months old, he's undergone his initial "stage 1, norwood" and then at 4-6 months of age had his stage 2 glenn surgery....

Unfortunately, at his age he would likely require some anesthesia which can be difficulty given his glenn flow...

I'd discuss the timing and risks with his primary pediatric cardiologist. ...Read more

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What are the surgeries to fix hypoplastic left heart syndrome?

What are the surgeries to fix hypoplastic left heart syndrome?

Norwood/Glenn/Fontan: A three-step surgical repair approach has improved outcomes for affected babies, however the intra- and inter-procedure mortality remains significant and the long-term prognosis guarded. See: http://texaschildrens. Org/locate/doctors/ayres, -nancy/. ...Read more

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What is the treatment for hypoplastic left heart?

What is the treatment for hypoplastic left heart?

Pediatric cardiologi: This is too complex for quick answer. See pediatric cardiologist or contact a pediatric cardiology clinic. Hrs www, thepmc. Org. ...Read more

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Is? Hypoplastic left heart syndrome usually fatal?

Is? Hypoplastic left heart syndrome usually fatal?

Frequently.: At least 20% mortality is expected with this diagnosis. If diagnosed in utero, termination of pregnancy should be offered <24 weeks. A 3-step sequence of major cardiac surgeries is nowadays the standard of care and has fairly good outcomes at experienced centers (norwood/glenn/fontan), with significant intra- and inter-procedural mortality. Http://texaschildrens. Org/locate/doctors/ayres, -nancy/. ...Read more

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What surgeries may be performed on a child with hypoplastic left heart syndrome?

What surgeries may be performed on a child with hypoplastic left heart syndrome?

Several.: A series of complicated cardiovascular operations are necessary to manage hlhs, with significant intra- and inter-procedure mortality. I think a consultation with an experienced pediatric cardiologist and pediatric cardiovascular surgeon in a center of excellence for congenital heart disease can only answer your questions in truth. Http://texaschildrens. Org/locate/doctors/ayres, -nancy/. ...Read more

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Which other surgeries may be performed on a child with hypoplastic left heart syndrome?

Which other surgeries may be performed on a child with hypoplastic left heart syndrome?

See below: There are usually a series of operations that are performed on these children to improve blood flow throughout the body. These patient can also require later surgery to increase the size of the shunts and conduits as the body grows in size. Talk to your surgeon to discuss the future operations that will need to be performed. ...Read more

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Is? Hypoplastic left heart syndrome genetic?

Is? Hypoplastic left heart syndrome genetic?

Yes - multifactorial: 2-6% recurrence risk with 1-2 prior affected siblings and up to 25% recurrence risk in familial autosomal recessive rare cases. ...Read more

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Can you tell me if I could go on a really fast roller coaster with hypoplastic left heart syndrome?

Can you tell me if I could go on a really fast roller coaster with hypoplastic left heart syndrome?

Best consult Ur doc: The limits to your activities are best determined by the physicians who know you the best. The general status of any complex congenital heart patient varies considerably from patient to patient. It also varies with each case at different points within their repair and rehabilitation. ...Read more

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How hard is it to diagnose? Hypoplastic left heart syndrome during? Pregnancy?

How hard is it to diagnose? Hypoplastic left heart syndrome during? Pregnancy?

Tricky.: Hlhs should be blatantly obvious in the third trimester, if one looks. However, most fetal anatomical surveys are performed at 18-21 weeks, and the full picture of this syndrome may not be esily appreciable at that early point in fetal development. So, in direct answer to your question, hlhs can be missed in over half the cases, unless a late third trimester cardiac evaluation is performed. ...Read more

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Is Surgery treatment for Hypoplastic left heart syndrome effective? Drs here (Jordan) said that the surgery can only give the child few extra days?

Is Surgery treatment for Hypoplastic left heart syndrome effective? Drs here (Jordan) said that the surgery can only give the child few extra days?

Maybe there?: My oldest HLH patient is now a married 27 yo cardiac clinic nurse. Her road to health has been complex, and it was truly the skill of a lot of fine surgeons and cardiologists that kept her going when things looked bad. She was on the transplant list for a while. Thirty years ago, we were at the point where your doctors seem to be. ...Read more

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My daughter was born with hypoplastic left heart syndrome and not diagnosed by ultrasound. Why not?

Screening ultrasound: Most probably, you had routine screening ultrasounds by your obstetrician during your pregnancy. Severe forms of hypoplastic left heart syndrome (hlhs) may be picked up by experienced obstetricians during their routine studies, but less severe forms may not. If the obstetrician suspects heart disease, he/she will refer you to a pediatric cardiologist for a complete fetal echocardiogram prenatally. ...Read more

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How to choose where to have a baby with hypoplastic left heart syndrome who will require a norwood procedure: need detailed source of info on costs, survival rates, etc at various hospitals.

How to choose where to have a baby with hypoplastic left heart syndrome who will require a norwood procedure: need detailed source of info on costs, survival rates, etc at various hospitals.

Tough decisions: I'm sorry that you have to deal with this and wish you the best. There are not that many centers that do this. Costs will depend on your insurance and the hospital you pick. As far as survival, these procedures are very rare. You can ask for the number they do and their survival rates. They should give that to you. For hplhs heart transplant is often needed. Good luck and take care. ...Read more

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My son has hypoplastic left heart syndrome. His liver is enlarged and his abdomen is distended. Is this due to diastolic dysfunction?

My son has hypoplastic left heart syndrome. His liver is enlarged and his abdomen is distended. Is this due to diastolic dysfunction?

Perhaps: There are multiple causes of liver enlargement and abdominal distention following fontan (i am assuming that he has had a fontan procedure.) my may have heart failure, diastolic dysfunction, obstruction in the pulmonary arteries, obstruction in the fontan circuit, or tricuspid insufficiency. Also, these findings may be signs of protein losing enteropathy. He needs a careful evaluation. ...Read more

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What is hypoplastic right heart syndrome?

What is hypoplastic right heart syndrome?

Hypoplastic Right: A congenital heart abnormality with an underdeveloped right atrium and ventricle. ...Read more

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I am 18 with hypoplastic right heart syndrome what are some health issues that Are in my adult years I should be aware of?

I am 18 with hypoplastic right heart syndrome what are some health issues that Are in my adult years I should be aware of?

Hard to say: The best answers for your question would come from your treatment team. Your diagnosis is not a "one size fits all" category and some do quite well after repair, some get new hearts (another problem) and some are quite restricted. At 18 you need to ask about your transition to adult cardiologists and begin that process. ...Read more