“Pink or blue?” That was the only question on their minds when parents-to-be Casey and Charles Clark visited her OB/GYN office for a routine ultrasound. They were thrilled to learn they were having a boy—but weren’t ready for the results that would forever change their lives.
A rare condition
The technician couldn’t see all four chambers of their baby’s heart. “Little did we know that the excitement would quickly turn into days of anticipation, waiting to find out what was wrong with Murphy,” says Charles. A fetal echocardiogram at Sanger Heart & Vascular Institute helped confirm that Murphy had a rare congenital heart defect called hypoplastic left heart syndrome (HLHS). The Clarks met with James Renè Herlong, MD, and Chief of Pediatric Cardiovascular Surgery Benjamin Peeler, MD, FACS. The Clarks were concerned about the road ahead, but their new team of caregivers gave them the confidence they needed to continue with Murphy’s treatment.
Dr. Peeler explained that a planned three-part reconstruction of Murphy’s cardiovascular system was necessary to correct the defect. The first surgery, the Norwood procedure, is typically performed within the first few days after birth. “It’s a challenging procedure, in which the aorta is enlarged and a small tube is placed from the right ventricle to the lung’s blood vessels,” says Dr. Peeler, whose Norwood survival rate is 100 percent for the 17 surgeries performed since he’s been treating HLHS patients at LCH.
Little Murphy’s first surgery took place only six days after birth, and he immediately showed amazing progress. “The doctor told us to expect a four- to-six-week hospital recovery,” said mom Casey, “but Murphy was discharged after only 10 days.”
Murphy returned to Levine Children’s Hospital for his second procedure and stayed for seven days. “His oxygen level has improved which makes him feel much better,” said Casey. After Murphy’s echo-cardiogram to check his heart function, everything looked good. “We’re so thankful for how well he has done so far,” says Casey.
“We can’t thank Dr. Peeler and the LCH staff enough,” added Murphy’s father, Charles.
What is HLHS?
Hypoplastic left heart syndrome (HLHS) accounts for 9 percent of all congenital heart defects. It’s more common in males than in females. The right side of the heart is normal, but the left side is underdeveloped. Because the aorta, left ventricle and valves are too small, the left side of the heart can’t send enough blood to the body. The right side of the heart has to perform the function of both sides, pumping blood to the lungs and out to the body via a patent ductus arteriosus (PD A). Fetuses depend on the PD A, but it typically closes in the first days of life. As a result, severely diminished blood flow to the organs causes the infant to go into profound shock. Without treatment, HLHS is fatal within the first hours or days of life.
“Prior to 1980, there was no effective treatment for HLHS, and unfortunately, babies typically died within the first month of life,” says pediatric cardiovascular surgeon Benjamin Peeler, MD, FACS. “But the Norwood procedure allows the right side of the heart to effectively do the work of both sides.”
Click here to learn more about the congenital heart program at Levine Children’s Hospital.