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.
2-6% with 1-2 sibs. Multifactorial inheritance usually is quoted for hlhs, with a 2% recurrence risk if one prior affected sibling and a 6% recurrence risk with two prior affected siblings. Rarely an autosomal recessive form can be seen with 25% recurrence risk from the same parents.
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.
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.
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.
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/.
HLHS Surgeries. Norwood (stage i) glenn (stage ii) fontan (stage iii) some programs may do a hybrid version of stage i, called the "hybrid stage i". Occasional patients with hlhs will need other procedures depending on their anatomy, but those 3 are the standard surgical treatments.
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.
Is Surgery treatment for Hypoplastic left heart syndrome effective? Drs here (Jordan) said that the surgery can only give the child few extra days?
Complex. Surgery needs to be performed in a highly specialized center for cardiac surgery, it is a complex surgery involving several stages, or ultimately a heart transplant, either way, life long follow up is needed, so unless the funds and other support means are available, as social/emotional support, life won't be easy for the child or the family, good luck.
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.
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/.
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.
Tertiarycare hospi. Choose nearest university hospital with nicu with reputable peds cardiac surgeons with experience.
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.
Ask. If you have a fetal echo to diagnose prenatally this condition, the physician counseling you about the diagnosis is a good place to start asking these questions. And do ask these questions.
Great question. One of the most valuable resources for parents of a child with hypoplastic left heart syndrome is an organization called sisters by heart. They have great parent resources to help parents develop expectations and help to learn about the important factors to help your baby as healthy as possible. Http://www. Sisters-by-heart. Org.
Begin by connecting. With a resource group in your area, to talk to others who have faced a similar challenge. Support groups are available online, at sites such as http://tchin. Org/index. Htm. Speak to different doctors, surgeons, and families, as each may have helpful opinions and information. Your fetal cardiologist (doctor who read the echocardiogram and provided this diagnosis) should be able to help as well.
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.