Imagine being told during your eighth month of pregnancy that your baby has a fatal heart defect and there is absolutely nothing anyone can do about it. It happened to Sandra and Marty Hinderliter when they went in for an ultrasound. Sandra's previous ultrasounds had been normal, so they were completely unprepared for the devastating news.

"We were told that the baby's heart had not formed," Marty Hinderliter recalls. "They said Sandra would have to carry the baby to term, and that the baby would die shortly after birth." The Hinderliters decided to get a second opinion at The CHOC Heart Institute. There, they met pediatric cardiologist Michael Rebolledo, M.D., who performed a fetal echocardiogram and diagnosed their baby's congenital heart defect. Fetal echocardiography is an advanced form of ultrasound imaging that allows physicians to visualize the inside of a baby's heart before birth.

Dr. Rebolledo came back with much better news for the Hinderliters.

"We found out exactly what our baby's heart problem was 'tricuspid atresia,' Marty recalls. "Dr. Rebolledo told us that our baby could be born, and then he explained the series of three surgeries that would be needed over the next few years to fix it."

The Hinderliters had come to the right place for a second opinion. The CHOC Heart Institute is the only pediatric cardiology program in Orange County offering open-heart surgery for children born with heart defects. This program is staffed with a team of dedicated pediatric-trained specialists, including cardiac surgeons, cardiologists, intensivists, anesthesiologists and cardiac nurses.

Congenital heart defects occur in six to eight out of every 1,000 live births. In Zoe's case, the tricuspid valve between the right atrium and right ventricle failed to develop properly. Without this valve, blood cannot flow into the right ventricle.

Surgical repair cannot be performed until after birth, but having an accurate prenatal diagnosis is important for developing a treatment strategy.

"Prenatal diagnosis is the first step in the comprehensive management of babies with heart defects. By knowing about congenital heart disease before birth, serious medical consequences can be avoided," says Dr. Rebolledo, who adds that a prenatal diagnosis also allows parents to be educated and counseled about birth defects. "In Zoe's case, a second opinion was very important. When confronted with a heart defect in a baby, it is important to get a second opinion from an expert pediatric cardiologist."

The CHOC Heart Institute works closely with obstetricians, perinatologists, neonatalogists, and families to coordinate care before and after birth. The Hinderliters chose to deliver at St. Joseph Hospital, in Orange, so their baby could be immediately transported next door to the CHOC Neonatal Intensive Care Unit (NICU).

Zoe Hinderliter arrived on September 16, 2000. Everything was ready for her, and all went as planned. Four days later, she had the first of the three planned surgeries to repair her heart defect. Pediatric cardiac surgeon Richard Gates, M.D., inserted a Blalock-Taussig shunt, a small tube, into her right pulmonary artery to re-direct blood into her lungs to increase the level of oxygen in her blood. Afterward, Zoe spent a month in the CHOC NICU and went home.

At 6 months, Zoe was ready for the second heart surgery. Dr. Gates performed a Glenn procedure that allowed blood from Zoe's upper body to flow directly into her lungs without having to be pumped there by her heart. This avoided putting extra strain on her heart.

Once again, Zoe did well, and for the next couple of years, she returned to CHOC periodically for follow-up visits and monitoring. She continued to grow, but her parents noticed that Zoe seemed to tire more quickly than her playmates.

The third and final corrective surgery for tricuspid atresia is the Fontan procedure, which usually takes place between the ages of 3 and 4. This procedure allows all the venous blood returning from the body to bypass the heart and enter the lungs to pick up oxygen before being pumped back to the body. Last April, Zoe was ready for it.

Zoe's recovery after her third surgery required an extended stay in the Pediatric Intensive Care Unit. All of the issues associated with a complex surgery of this type were successfully managed and resolved by the expert physicians, surgeons and nurses at The CHOC Heart Institute.

The surgical repair strategy that began when Zoe was 4 days old is finished. Zoe continues to receive regular check-ups at The CHOC Heart Institute, but she has no physical restrictions and can look forward to a normal childhood.

"Children undergoing all three stages of this surgery have a 98 percent chance of survival," Dr. Gates says. "The majority of these kids will be able to play like other kids and will be indistinguishable from their peers."

The Hinderliters shudder to think of what might have happened if they hadn't gone to CHOC for a second opinion. "If we'd gone with what that first doctor told us, this story would not be happening," Sandra says. "Always get a second opinion."

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