Life-saving surgery rebuilds the tiniest hearts
Natalie and Ernie Hance of Blackwood expected to see what all parents expect to see during a fetal ultrasound - hands, feet, a beating heart and the baby's sex. Instead, they found out their baby had hypoplastic left heart syndrome (HLHS). Previously thought to be lethal, it is a rare and complex congenital heart defect that now has a much better prognosis with surgery.
Finding the problem
A team approach
Complex surgery
Rapid treatment to save a fragile heart
Nicholas today
Finding the problem
The diagnosis came shortly after a heart abnormality was picked up during a routine ultrasound to measure the baby's growth and development at 20 weeks. Natalie Hance was referred by her doctor to the antenatal testing unit at Virtua West Jersey Hospital Voorhees, where she received a higher level ultrasound. Ronald Librizzi, DO, chief of maternal-fetal medicine at Virtua Health, confirmed the diagnosis and ordered a fetal echocardiogram, a heart study that uses sound waves to produce detailed images of the heart and its function.
Dennis Wood, RDMS, RCPT, performed the heart study and confirmed that the fetus had an abnormal heart. He arranged for the family to meet with a pediatric cardiologist. "To help parents better understand what I'm telling them about their baby's heart," says Wood, "I give them drawings of both a normal heart and a simplified version of the abnormal heart for comparison. I also tell them that they are now part of our family."
A team approach
When a fetal abnormality is diagnosed prenatally at Virtua, patients are treated by obstetricians, maternal-fetal medicine specialists, neonatologists and other pediatric subspecialists, as well as nurses and technicians.
Through this team, Hance met Samuel Gidding, MD, Virtua/duPont pediatric cardiologist. Dr. Gidding explained HLHS and how it is treated: "The main problem in this defect is that the left ventricle and the first part of the aorta are underdeveloped. After birth, the baby's heart can't pump enough blood to the body. The primary option for treatment is a series of three operations the first of which is known as the Norwood procedure."
Complex surgery
The goal of the Norwood procedure is to utilize the functional right heart to pump oxygenated blood to the body and simultaneously direct deoxygenated blood directly to the lungs. The first stage is performed in the first week of life; the second is performed at around 6 months; and the third and final is performed at around 12 months. Each stage reconstructs different parts of the heart so it can function as normally as possible.
What reassured the Hances about their newborn's impending surgery was that it would be performed by William I. Norwood, MD, the nationally renowned pediatric cardiothoracic surgeon who created the life-saving procedure. The surgery would take place at the Nemours Cardiac Center at the Alfred I. duPont Hospital for Children in Wilmington, Delaware. "It was quite a relief to meet Dr. Norwood before the surgery. We toured the center and met with a social worker who explained what to expect during and after the surgery and answered our questions. It was all very uplifting," said Hance.
Rapid treatment to save a fragile heart
Hance delivered her son Nicholas on November 29, 2002. After being stabilized by the neonatal care team, Nicholas was whisked off to the Nemours Cardiac Center in Wilmington with his father and a specially trained pediatric care team.
On December 2, Dr. Norwood completed the first stage of surgery on little Nicholas. "During the two-and-a-half hour surgery, a surgical coordinator updated us often on the progress," said his mother. "Afterward, my husband and I stayed right in the room with Nicholas during his two-week recovery in the hospital. The medical staff took great care of Nicholas and us - they really took a weight off our shoulders."
Nicholas today
Nicholas now visits Dr. Gidding in Voorhees every six weeks in preparation for the second stage of his surgery. Dr. Gidding monitors Nicholas' weight, pulse and heart function to make sure he's progressing normally. He also reassures the Hances that Nicholas has a great future ahead of him: "He may not be an athlete, but he could be a brilliant pediatric cardiologist."