Hypoplastic Left Heart

Le Bonheur offers comfort, hope from heart diagnosis

It’s rare to miss a prenatal Hypoplastic Left Heart Syndrome diagnosis. But that was little comfort Saralyn and Mark Crowell. The Crowells had expected a normal birth of their first child, Jack. Instead, shortly after his delivery, they were learning about Hypoplastic Left Heart, a congenital defect that causes the left ventricle of the heart to be underdeveloped. He would need three surgeries to reconfigure blood flow to his heart.

At six days old, Jack underwent the first of those surgeries, a Norwood, which involves atrial septectomy and transection and ligation of the distal main pulmonary artery.

In a field of medicine where outcomes matter most, the Crowells rested easy know that Knott-Craig touted a 90 percent survival rate for the Norwood operation – far better than the Society for Thoracic Surgeons (STS) national average.

STS recently gave Le Bonheur’s Heart Institute the highest possible three-star rating. Le Bonheur is one of only 10 of the STS’s 117 participating programs to receive the rating in the most recent STS Congenital Heart Surgery Database Feedback Report.

“Those first four months, we worried and were so concerned,” said Saralyn. “Drs. Knott-Craig (Jack’s surgeon) and Ballweg (Jack’s cardiologist) gave us their phone numbers and helped us through that period. They were a huge security blanket.”

At 4 months of age, Jack underwent the second surgery – a bi-directional Glenn – to divert half of the blood to the lungs, as circulation through the lungs no longer needed as much pressure from the ventricle.

Jack’s shunt to his pulmonary arteries was disconnected, and his right pulmonary artery was connected directly to the superior vena cava.

Finally, this past summer, when Jack was 2 ½ years old, he underwent the final procedure, a Fontan. In the Fontan, surgeons connected the inferior vena cava to the pulmonary artery by creating a channel to direct blood to the pulmonary artery. Now, all deoxygenated blood flows through the lungs passively.

One month post-surgery, Saralyn said she has already noticed Jack’s appetite and energy improving.

The Crowells benefited from a growing Le Bonheur Children’s Heart Institute team focused on transforming the way it cares for children and adults with congenital heart defects by providing a depth of talent, technology and research.