30 April 2009

Valves go with ventricles

Hypoplastic Left Heart Syndrome (HLHS).

HLHS is a congenital cardiac malformation that falls under the heading of "single ventricle" defects. Essentially, the baby is born with only one functioning ventricle that must supply (actively or passively) both the body and the lungs. They also fall under the coloquial heading of "blue babies."

Typically HLHS babies will have a poorly formed left ventricle, mitral valve, aortic valve and proximal aorta. In order to promote delivery of blood to the body, the ductus is kept open by administering prostaglandins and in some cases the foramen ovale will be enlarged (in the cath lab or the OR).

Surgical repair for this condition is done in three stages: a Norwood procedure (with central shunt) shortly after birth, a hemi-fontan or bi-direction Glenn a few months later and eventually a full fontan at 2-3 years. Pictures and explanations of those in a later post. Sometimes the right heart cannot cope with the strain and transplantation needs to be considered.

HLHS occurs more frequently in boys than girls and has an overall prevelence of roughly 4 in 10,000 live births.

On a more personal note, a child I visited often in the hospital made it to her hemi-fontan, but her heart couldn't cope with the stress. She was on the transplant list for months and recently received her new heart. I was able to watch them close her chest three days post-op (in major cardiac surgery on infants they generally delay sternal closure) and she is growing stronger daily.

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