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Tom Smart, Deseret Morning News
Camille Hood watches as sonographer Lynn Gennett monitors her unborn baby's heart Wednesday at Primary Children's Medical Center.

They haven't yet picked his name, but Camille and Gary Hood know a great deal about their baby boy, who is due in May. They know he has a complicated set of heart defects and his early future will include close monitoring and staged surgeries.

He has what's called tricuspid atresia, and his aorta and pulmonary artery are reversed. The tricuspid valve on the right side of his heart didn't form, so the blood doesn't flow as it should from the upper right chamber into the right ventricle, which is missing. The blood flow to his lungs is wrong, as well, but inside his mother's womb, little holes between chambers help him survive until his heart can be repaired.

Wednesday, the Hoods, who live in Woods Cross, went to Primary Children's Medical Center for a new ultrasound, then met with Dr. Michael Puchalski, a pediatric cardiologist specializing in imaging, to talk about what they can expect and what will be needed once the baby is born.

Puchalski, Dr. Jason Su, also a pediatric cardiologist, and nurse Kelly Kelso have established the Fetal Heart Center at Primary Children's Medical Center. It's a program that aims to detect heart defects before a baby is born to be sure immediate and appropriate care is available.

Many, but not all of the detected defects require urgent and immediate care. Knowing — and making plans — saves tiny lives, says Puchalski. Before they formed the center, he says, prenatal doctors in the Intermountain region were only detecting about one-third of heart defects prior to birth. That number has grown to about 55 percent, in part because of intensive outreach from the trio, who have worked with obstetricians and even invited sonographers up to train to recognize nuances of less obvious defects. They've done grand rounds at area hospitals and created handouts to raise awareness.

Puchalski says he'd like to see early diagnosis in at least 70 percent of cases.

Knowing about heart disease in utero "can prevent deaths," Puchalski says. Because the babies will be cared for at the children's hospital, some parents who expect their baby will require urgent care may choose to deliver nearby at University Hospital. For others, arrangements can be made for transport to Primary. What they're trying to avoid happens all too often: A child is not diagnosed until symptoms appear, sometimes after parents have taken home their "healthy" newborn, and they're faced with a huge shock and need for unexpected emergency intervention.

"It's given us more time to emotionally and financially prepare. We've had some time to get ready for what's coming," says Gary Hood, who is a nurse practitioner specializing in adult cardiology.

For Camille, a nurse at University Hospital, the Fetal Heart Center has also put all the consultations in one place, reducing stress and coordinating things.

Kelso routinely takes parents expecting a child with a serious heart defect on a tour of the intensive care unit, so they'll know what it looks like and what to expect. They talk to parents about the surgery — or surgeries — that will be needed and what will be done. In a very rare case, a baby had his surgery in utero.

In Baby Hood's case, they can't plan everything yet. Variables like how large the ascending aorta is make a difference. If it's a good size, immediate intervention may not be needed. Typically, the three-phase surgery for tricuspid atresia starts with a shunt when baby's pretty young to connect the aorta to the branch pulmonary artery, so blood can flow to the lungs. In phase 2, a Glenn shunt, the main pulmonary artery is tied off and removed, then the superior vena cava is connected to the right pulmonary branch artery, the unoxygenated "blue" blood of the upper body sent to the lungs without passing through the heart. At that stage, the earlier shunt is removed. The Glenn shunt reduces how hard the heart has to work.

Finally, in a Fontan procedure when the baby is 2 or 3, the blue blood from the lower part of the body will be sent to the lungs using the inferior vena cava, the right atrial wall and Gore-Tex material. It too enters the lungs without being pumped by the heart. The heart's job will be to pump red, oxygenated blood out to the body.

Besides Mom and Dad, the baby has a brother, Sam, who is 3.

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