Single Ventricle Anomalies
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Hypoplastic Left Heart Syndrome (HLHS)
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This web site is not intended as medical advice.  Please consult your physician with any questions you may have regarding your child's condition.

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There are other single ventricle heart defects that require similar surgeries to HLHS - either a heart transplant or staged reconstruction resulting in the Fontan circulation.  If your child is diagnosed with one of these other types of single ventricle defects, much of the site will be applicable to you.  In some cases, the surgeries may vary slightly from the HLHS surgeries, depending upon the diagnosis, so discuss with your surgeon what treatment is necessary for your child's specific heart condition.  Please see the information below for the following conditions:

Hypoplastic Right Heart Syndrome (HRHS)
Tricuspid Atresia
Double Inlet Left Ventricle (DILV)
Double Outlet Right Ventricle (DORV)
Single Ventricle

Hypoplastic Right Heart Syndrome is a condition in which the right side of the heart is underdeveloped including the right atrium, and often the pulmonary and/or tricuspid valves. With HRHS, the baby's stronger side of the heart - the left side - is functional.  The right side of the heart usually pumps blood to the lungs to be oxygenated.  When the right side of the heart is underdeveloped, it is not able to get blood to the lungs.  Babies born with this condition are OK at first, because the patent ductus arteriosis (PDA) is a connection that allows blood to go to the lungs.  In all babies, this PDA usually closes within the first few hours or days of life.  Once this connection closes, a baby with HRHS will not be able to get blood to the lungs to be oxygenated. 

The treatments for HRHS are similar to that for HLHS babies.  Prostaglandin is usually administered once the baby is born to keep the PDA open.  Children with HRHS usually undergo 3 surgeries, with the first one being a BT shunt (and not the Norwood like with HLHS.) In some cases, the BT shunt will not be necessary, and a PA banding (pulmonary artery banding) will be done instead.  The second and third stage surgeries are usually the same as for HLHS - the Glenn (or Hemi-Fontan) around 6 months to a year of age, and then a fontan surgery around age 2-5.  (The timing of the surgeries can vary depending upon your child's condition, so ask the surgeon when each stage would be completed.) 

Because the surgeries are similar, many parents of children with HRHS join support groups for HLHS.  There is also an HRHS online support group specifically for parents of children with HRHS.  

To read more about the treatment for HRHS, see the University of Chicago's website.   
For good diagrams showing HRHS, see The Heart Institute for Children (THIC) - and the Children's Heart Institute.

The tricuspid valve in a normal heart allows blood into the right ventricle from the right atria.  When there is tricuspid atresia (absence of this valve) blood cannot flow into the right ventricle as it is meant to, and the right ventricle is usually underdeveloped leading to hypoplastic right ventricle or hypoplastic right heart syndrome.

See the following links for more information on Tricuspid Atresia:
Cincinnati Children's Hospital
Lucille Packard Children's Hospital
Heart Center Online

Cove Point Foundation
Open Heart site

Heart Center Online
Cove Point Foundation

Cincinnati Chilldren's Hospital
CovePoint Foundation

If your child has been diagnosed with one of these single ventricle anomalies, most of the site will be applicable to you.  Please see the Questions to Ask to help you find a surgeon and hospital.  Having an experienced surgeon perform these complicated surgeries is very important in treating any of these very complex heart conditions.

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