Hypoplastic Right Heart Syndrome

Caleb has a condition called "hypoplastic right heart syndrome." Here is some information about it.

HYPOPLASTIC RIGHT HEART SYNDROME (HRHS)

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 the child's condition.)