Two year-old Hezekiah Moore loves to play his toy drum, keyboard and guitar. He greets strangers with a handshake and can turn a room upside down with his ebullient personality.
Hezekiah’s parents, Thomas and Tawana Moore of Virginia Beach, consider their toddler’s high energy a blessing because they came dangerously close to losing their only son when he was just a few weeks old.
Tawana first learned of a potential problem when she was four-and-a-half months pregnant in 2001. After a routine ultrasound, her obstetrician told her something might be wrong with the baby’s heart.
He sent Tawana to maternal-fetal medicine specialists for an evaluation. It was there that the Moores first heard the odd-sounding term “truncus arteriosus.”
“We had never heard of it,” she said. “The doctor told us that truncus arteriosus is a serious heart condition, and our baby might be really sick and was going to need surgery.”
The specialist sent the Moores to pediatric cardiologist Daniel Schneider at Children’s Hospital of The King’s Daughters for additional tests to determine the extent of the unborn baby’s defect.
Dr. Schneider performed another ultrasound for a good look at the baby’s heart. It confirmed the diagnosis.
“The quality of ultrasound images these days is so good that we can determine in advance of delivery that there is a problem with the heart,” he said. “In this case, we were fairly certain that Mrs. Moore’s baby did indeed have truncus arteriosus.”
Dr. Schneider explained to Thomas and Tawana that truncus is a congenital defect found in one of every 10,000 live births. It’s an abnormal development of the fetal heart that occurs during the first eight weeks of pregnancy.
Using diagrams and illustrations, he explained how the heart begins as a hollow tube, and the chambers, valves and great arteries develop throughout those first eight weeks. The aorta and pulmonary artery start as a single blood vessel, which eventually divides and becomes two separate arteries.
Sometimes, the single great vessel fails to separate completely, leaving a connection between the aorta and the pulmonary artery. This is the essential problem in truncus arteriosus. The disorder is accompanied by an opening between the right and left ventricle called a ventricular septal defect. Blood from the ventricles mixes as it leaves the heart, causing varying degrees of cyanosis – a blue coloring of the skin, lips and nail beds.
“Babies with truncus and most heart defects do fine during the course of the pregnancy,” Dr. Schneider said. “It’s usually not until sometime after delivery that symptoms appear.”
But knowing ahead of time that their baby had a heart defect helped the Moores prepare for what was to come.
“We knew the baby would need surgery and that it would be fairly extensive,” Tawana said. “Almost immediately after getting the diagnosis, we contacted our pastor and our church to ask everyone to pray for our baby. We had faith that everything would be okay.”
Despite the number of ultrasounds and exams she’d had, Tawana and her husband still wanted to be surprised about the sex of their second child. She did everything possible to carry the baby to full-term and wondered if a boy or a girl would be joining them and their then 12-year-old daughter, Jalease.
Arrangements were made for Tawana to deliver at Sentara Norfolk General Hospital because of its proximity to CHKD. The family was given a tour of CHKD’s Neonatal Intensive Care Unit to see where their baby would spend his or her first few days of life.
To Thomas and Tawana’s delight, Hezekiah Marcellus Moore was born full term on December 14, 2001, and weighed 9 pounds, 7 ounces. “He moved his arms and legs a lot and cried just like all new babies do,” Tawana said. “On the outside, he seemed like a perfectly healthy baby.”
After his parents held him for a few minutes, Hezekiah was taken to CHKD’s NICU for evaluation by the neonatologists and cardiologists. He needed supplemental oxygen for a few days but showed no signs of illness yet.
Surgery to repair his heart defect was scheduled for January 7, 2002, so Hezekiah could go home with his family for a couple of weeks.
“With truncus, it’s necessary to delay surgery until the child is out of the early neonatal period because lung pressure is extremely high during those first few days after birth,” said Glenn Barnhart, the cardiovascular surgeon who performed Hezekiah’s open-heart surgery at Children’s Hospital when he was 3 weeks old.
For 87 minutes of the six-hour surgery, Hezekiah’s heart was stopped so the surgeon could access the affected arteries and make the repair. During that period, a heart-lung machine provided temporary circulation of blood and oxygen to the boy’s other organs and tissues. Using a cardiac cath as a “road map,” Dr. Barnhart carefully detached the pulmonary artery from the common truncal vessel and connected it to the right ventricle using an allograft – an intact section of pulmonary artery from a tissue donor. He then closed the baby’s ventricular septal defect with a patch.
Thomas and Tawana were thrilled when they learned that surgery had gone well and that Hezekiah was in the Pediatric Intensive Care Unit recovering. But soon after, their baby’s blood pressure dropped and Dr. Barnhart took him back to the operating room.
“I fell apart,” Tawana remembers. “But it took only an hour for Dr. Barnhart to repair the bleeding that caused the problem.”
“I remember the first time we saw him after surgery,” Thomas says. “There were tubes and wires all over his little body. He was sedated and looked so helpless. I wanted to take his place and bear all the pain for him.”
After a period of time in the Pediatric Intensive Care Unit, Hezekiah was then moved to a regular hospital room on the eighth floor to continue his recovery.
“We were so anxious to take him home,” Thomas said. “But we were confident that the doctors wouldn’t send him home until they were certain he was ready.”
That day came on March 11. Hezekiah was discharged with a feeding tube and supplemental oxygen. The Moores were trained by CHKD nurses to properly care for him.
“I wasn’t nervous having him home,” Tawana said. “We were just so happy to finally have him there with the rest of the family.”
Hezekiah is now free of all medications and has no restrictions on his activities. He’s taken swim classes and goes to child care with other children his age. Pediatric cardiologist Michael Vance has performed subsequent cardiac catheterizations to stretch the allograft and the pulmonary arteries. And according to Dr. Barnhart, he will need another open-heart surgery when he is 5 or 6 years old to replace the allograft with a larger one as he grows.
But life for the Moores now is like that of most Hampton Roads families – school, work, church, activities. And lots of time spent loving Hezekiah.
“He’s our miracle child,” Tawana said. Thomas added, “We believe that everything happens for a reason, and in this case, Hezekiah’s illness has brought us closer as a family.
“If it weren’t for CHKD, he may not have been here at all.”
Drs. Schneider and Vance practice with Children’s Specialty Group PLLC at Children’s Hospital. Dr. Barnhart practices with Mid-Atlantic Cardiothoracic Surgeons and is director of cardiovascular surgery at CHKD.
This story was featured in the second quarter 2004 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.