Hypoplastic Left Heart Syndrome: Francis’ Story
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Francis was born with hypoplastic left heart syndrome (HLHS), a severe heart defect. After the first two of a staged series of three heart reconstruction procedures, he is doing well. Now 1 1/2, he’s an active and happy toddler.
At birth, Francis appeared to be a healthy baby boy. He weighed almost 9 pounds. The next morning, his parents, Ashleigh and John, woke up to learn that he had a serious heart defect.
Francis had a pulse oximetry test, which measures oxygen levels in a newborn’s blood. Screening is mandatory in Pennsylvania where Francis was born, and infant deaths from critical heart defects have declined by 33 percent in states where screening is required by law.
The results showed that Francis’ heart wasn’t circulating oxygen to all parts of his body. An echocardiogram when Francis was 10 days old confirmed that he had critical aortic stenosis and hypoplastic left heart syndrome (HLHS).
Ashleigh and John were told that Francis would need to be transferred to a hospital with expertise in treating infants with congenital heart disease. They chose the Cardiac Center at Children’s Hospital of Philadelphia (CHOP), almost two hours away from their home in Kunkletown, PA.
Ashleigh had worked as an EMT and was familiar with the depth of the Cardiac Center expertise. That afternoon, a cardiac transport team from CHOP came to bring Francis by ambulance to Philadelphia.
The next day, Francis underwent an interventional catheterization to dilate his aortic valve. He spent several days in the Cardiac Intensive Care Unit, and his care was led by cardiologist Chitra Ravishankar, MD.
Hypoplastic left heart syndrome is palliated with a series of three reconstructive surgeries, the first of which is typically performed in the baby’s first week of life. The second is typically performed around 4 to 6 months of age, and the third when the child is 2 to 4 years old.
Stephanie Fuller, MD, an attending surgeon in the Division of Cardiothoracic Surgery, performed the stage 1, or Norwood procedure, placing a shunt in Francis’ heart to control blood flow.
Francis remained at CHOP for 2 1/2 weeks after the surgery. During that time, Ashleigh and John received extensive training and education to prepare for his care at home. They learned how to manage his nasogastric (NG) feeding tube, and the signs to watch for that might indicate a need for urgent medical care.
After Francis was discharged, Ashleigh and John followed a careful regimen for his care, measuring his formula intake and weighing him every day. They reported the numbers to CHOP’s Infant Single Ventricle Monitoring Program (ISVMP), a program that provides specialized care for high-risk infants during the time between the first and second staged heart surgeries.
Francis gradually started to show signs that he was ready for the second surgery. At 4 months, he had the second of the staged heart operations, the Glenn procedure, which redirects the flow of deoxygenated blood from the upper body to the lungs, bypassing the heart.
Francis recovered well from the surgery, though he continued to show disinterest in bottle feeding. To make tube feeding easier, Francis’ medical team at CHOP replaced the NG tube with a gastronomy tube (G tube), which is surgically inserted through the abdomen to the stomach.
Now 1 1/2, Francis is a happy, active toddler. His favorite toy is a small indoor slide, which he climbs up and slides down again and again. He loves to play with his older sisters, especially with toy cars. And he has a special affection for Mickey Mouse.
Ashleigh and John take Francis for periodic follow-up visits to CHOP’s Cardiac Center, Allentown in PA, where he is seen by cardiologist Robert Palermo, DO, FACC. The family appreciates that they can see a cardiologist in their community while still having access to the cutting-edge resources of CHOP’s Cardiac Center, which is one of the world’s leading pediatric heart centers.
At some point in the near future, he will need the third and last of the staged heart reconstruction operations, the Fontan procedure, which will complete the rerouting of his circulation around his heart.
“Going to CHOP was the greatest decision we’ve ever made,” says Ashleigh.
“The doctors are awesome and the nurses couldn’t be better. They saved my son twice. We are blessed to have CHOP for Francis.”