Summer 2005

Twin Heart Defects Test a Family and Physicians

Signs that there might be trouble for fetal twins Arabella and Adriana Cipponeri were first spotted by an astute ultrasound technician at the Memorial Medical Center in Modesto.

Bad News

"I had been given about 10 ultrasounds before, so I didn't think much about it, but this one seemed to take about three times as long as the other ones," says mom Katie Cipponeri. The technician discussed the scan with obstetrician Sean Clare, M.D., who later that day gave Katie the bad news: One fetus, Arabella, had a serious heart defect called hypoplastic right heart syndrome. Usually caused by a blocked pulmonary valve, hypoplastic right heart syndrome involves a severe hypertrophy and stiffening of the muscles in the right side of the heart. A level II ultrasound done by another physician confirmed the diagnosis.

The Modesto physicians had done a good job in catching the problem early. A high percentage of heart problems are not diagnosed until after birth, when a baby is already in distress, says Lisa Hornberger, M.D., director of the Fetal Cardiology Program at UCSF Benioff Children's Hospital. Knowing about the problem gave Katie and Daniel Cipponeri and their physicians time to prepare.

But prepare for what? As Katie and Daniel learned the severity of the condition, their hopes dimmed. From the Modesto physicians and through their own research, they discovered that the most they could hope for their baby was a series of palliative operations that would at best result in a heart with a single working ventricle.

After learning a great deal about hypoplastic right heart syndrome, the Cipponeris came to the painful conclusion that if Arabella really had the disorder, they would not take heroic measures if it meant putting her through a series of surgeries that would keep her barely alive on half a heart.

Fetal and Neonatal Expertise

Told that UCSF Benioff Children's Hospital offered the greatest expertise in this disorder, the Cipponeris were referred to a consultation with Theresa Tacy, M.D. Tacy and her colleagues at UCSF's Fetal Cardiology Program, Hornberger and Michael Brook, M.D., each have over 10 years of experience doing fetal ultrasound screening, and often are able to find things that other doctors might miss.

"We have state-of-the-art equipment and have very strict standards for how to evaluate the cardiac structure and function," Tacy says. "We carefully take exact measurements of each valve and of blood velocities through the valves, for example."

A vast store of experience is also a crucial part of UCSF's diagnostic strength. Tacy's group gets 400 to 500 fetal cardiac referrals each year, and sees about 3,500 pediatric cardiac cases annually, she says.

After a complete workup, Tacy had both good news and bad news for the Cipponeris. The good news was that Arabella did not seem to have hypoplastic right heart syndrome, but did have a pulmonary atresia with a good-sized right ventricle. Learning that Arabella had a less serious problem gave them hope once again. "Dr. Tacy told us that from what she could see, there was no reason not to intervene," Katie said.

The bad news was that Arabella's fraternal twin, Adriana, also had a heart problem. For Adriana, the problem was stenosis of the pulmonary valve.

This is a much milder form of Arabella's problem. These obstructions in the blood flow from the heart to the lungs meant that Arabella — and possibly Adriana, as well — would have problems when the ductus arteriosus closed after birth.

"Without these diagnoses, we could have lost both babies," Katie Cipponeri says.

At UCSF Benioff Children's Hospital, Katie's case was followed by a spectrum of specialists: pediatric cardiologists, obstetricians, perinatologists, neonatologists, surgeons, nurses and social workers from the UCSF Birth Defects Center. Even after Katie returned home to Modesto, all worked together to make sure that when Katie gave birth, everything would be ready to give the babies the best possible chance to survive and thrive.

Back in Modesto, in her 32nd week of pregnancy, Katie's amniotic sac broke. The plan was to get Katie to UCSF as quickly as possible — but the plan didn't quite work. Weather grounded the helicopter, and mechanical problems delayed an ambulance. After Katie finally arrived at UCSF Benioff Children's Hospital, physicians discovered that one of the babies' umbilical cords was prolapsing. The doctor "hit a button on the wall and both babies were out in about four minutes," Katie says. Arabella came into the world at 3 pounds, 12 ounces; Adriana at 3 pounds, 4 ounces.

Medical Intervention

The UCSF Center for Mothers and Newborns neonatal intensive care team took over from the labor and delivery team moments after birth, speeding the babies to the Neonatal Intensive Care Nursery. After Katie's discharge, she and Dan stayed at UCSF's Koret Family House near the hospital, so they could spend time every day with their newborns.

Both babies were given prostaglandins to keep their ductus arteriosi open and to give the physicians more time to evaluate each infant. Adriana was put on CPAP and Arabella was intubated. In her second day of life, Arabella was given an interventional cardiac catheterization by pediatric cardiologist Phillip Moore, M.D., to open her pulmonary valve. A catheter was threaded up through her femoral vein to the right side of her heart, where a short, sharp jolt of radiofrequency energy burned a small hole in her pulmonary valve. Then a balloon catheter was inserted into the hole and inflated, opening up the valve's fused leaves.

A muscle hypertrophy right below Arabella's pulmonary valve still partially blocked blood flow, but the UCSF surgeons decided to wait until Arabella grew a little more before they performed surgery.

Adriana's valve problem was milder, and she was taken off oxygen and prostaglandin infusion without any difficulties. For the time being, she could be treated as if she had no heart problems.

After a month and a day in the hospital, the twins went home. Just before the end of 2003, Arabella was back at UCSF Benioff Children's Hospital, ready to have her first surgical procedure to remove the muscle mass impeding blood flow through her pulmonary valve. Adriana would get a balloon valvuloplasty in the catheterization laboratory to allow greater blood flow through the stenotic pulmonary valve. Katie Cipponeri sat down with pediatric cardiac surgeon Tom Karl, M.D., who explained the procedure he planned to perform on Arabella.

"He is really an incredible human being," Katie says about Karl. "I have never seen a surgeon of that caliber before, and he spent as much time as it took to explain everything. I was so impressed."

Karl performed the surgery in early January 2004, and Arabella came through the surgery with flying colors, Katie Cipponeri says. "She was extubated the next day and was home 10 days later. The catheterization and balloon valvuloplasty on Adriana also went well."

Happy Outcomes

By last summer, both babies were tipping the scales at 16 pounds apiece and growing rapidly. This year, they are walking, talking, playing and getting into mischief like any 21-month-olds.

The successful delivery and treatment of the twins, says Ian Adatia, M.D., medical director, Pediatric Cardiac Intensive Care Unit, are excellent examples of the way that specialists in many disciplines work together at UCSF Benioff Children's Hospital to create a good outcome. "This case required expert neonatal care and excellent surgical skills, as well as presenting a challenging diagnostic scenario and challenging care in the catheter lab," Adatia says.

For the grateful Cipponeris, the teamwork was a big part of turning what could have seen a tragic situation into a triumph. "The transitions (between teams) were just beautiful," Katie says. "Above all else, there is a level of humanity and compassion that I had not seen before.

"To go from possibly planning a funeral to thinking about a lifetime of wonderful memories, having two girls who hold hands when they sleep at night — I just can't say enough."

For more information, contact the Pediatric Heart Center at (415) 353-2008.

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