Kristine Rasmussen with her 6-week-old son Oliver, who received a pacemaker shortly after birth in April 2009. At right, Kristine's husband, Thomas, held Oliver's twin brother, Cameron.

A Matter of the Heart

Oliver Rasmussen has a congenital heart defect and could have died soon after birth in April 2009. But thanks to a Children's Hospital Oakland cardiology team, the pacemaker he received will let him live a normal life.

In the backyard of the Rasmussen home, pink roses planted in memory of Katja Rasmussen were dormant. It was Dec. 22, 2008, three years since her death at age 3 weeks. Her mother, Kristine, still felt the hurt; but she was also excited about her new pregnancy—twins.

Kristine was 24 weeks pregnant. On her way out the door for a routine visit with her perinatologist, she glanced at a photograph of Katja. Complications from a rare neurologic disorder had led to her death. In the photo, Katja has thick black hair—like her mother—and is breathing oxygen through nasal tubes.

As she drove from their home in Lafayette to the doctor’s office, Kristine also thought about her husband, Thomas. Like her, he is an airline pilot. Thomas was out of town that day, flying for a regional airline based in Indianapolis. This was the first appointment he’d been unable to attend and Kristine missed his support.

All was well during the appointment, until her perinatologist, Jeffrey Traynor, MD, noticed something unusual during a routine ultrasound. One fetus’ heart was beating normally, about 150 beats per minute. But the other fetus’ heart was only beating in the low 50s.

The suspected cause? Complete heart block. It had probably begun only a few weeks earlier, induced by abnormal maternal antibodies attacking and severing the electrical connection between the upper and lower chambers of the fetal heart.

Dr. Traynor immediately referred Kristine to the Cardiology team at Children’s Hospital & Research Center Oakland. He regularly works with Children’s affiliated subspecialists, particularly in neonatology and cardiology, is comfortable with them, and familiar with the teamwork needed to help mothers like Kristine.

“That whole cardiology group is great,” said Dr. Traynor, medical director of Maternal Fetal Medicine at John Muir Health. “The Children’s physicians take care of patients well.”

Fetal Medicine Service Starts before Birth

Children’s cardiologists are part of a fetal medicine service at Children’s that can begin care before birth; so ill newborns can be treated by Children’s subspecialists as soon as they’re born. In this instance, Howard Rosenfeld, MD, was the first Children’s cardiologist to see Kristine.

“This kind of program allows for the most seamless coordination of care, in this case, from the delivery to transport to pacemaker placement,” said Dr. Rosenfeld.

Because it was only days before Christmas, Kristine and Thomas had a week to fret before their appointment. “We hoped what Dr. Traynor heard was just a temporary condition,” said Kristine. “But there was no denying it. With the Internet, we could research it. It didn’t look good for us.”

Dr. Rosenfeld examined Kristine and confirmed that both fetuses had structurally normal hearts with no physical blockages, but one was in complete heart block. The upper chamber was sending the right signal, but the lower chamber was not receiving it.

The result was a “default” heart rate of about 53. Mortality rates for fetuses with heart rates less than 55 can be up to 50 percent.

The most common cause is a mother carrying certain abnormal antibodies, said Dr. Rosenfeld. Frequently she may be a mother with lupus. Kristine had few symptoms, but testing found that she was carrying the antibodies because she had Sjogren’s syndrome.

These antibodies can attack the fetal atrioventricular node at about 19 weeks gestation, permanently damaging the fetal heart’s ability to coordinate atrial and ventricular function.

“I love working with families living under these circumstances,” said Dr. Rosenfeld. “We can actually give them a lot of reassurance based on prior good outcomes with fetuses. It’s nice we could tell her we had a very similar situation with twins where both twins did well. The Rasmussens appreciated the amount of info we are able to give them. They liked knowing we had a plan in place for what we would do after their twins were born.”

While he and the rest of Children’s fetal medicine team developed their plan, the Rasmussens came to terms with their fears.

“It was a shock,” said Kristine. “Since Thomas was gone I had to go to Children’s Hospital by myself, and I almost had a total breakdown. I couldn’t believe I was there again, where Katja had been. The bottom of my world had just been removed and I was falling into a well.

“But that’s where our pilot training comes in. You can’t let your emotions run this. You have to think logically. You have a problem to solve; you can’t panic; you have to land the plane.”

Pilot Training Helped Them Cope

Airplane metaphors come naturally to Kristine and her husband. Originally trained to be a graphic designer, Kristine designed marketing materials for Sierra Academy of Aeronautics in Oakland while learning to fly there in her spare time.

The adventurous, motorcycle-riding designer worked her way through all the licenses, qualified to be a flight instructor, eventually became an airline pilot, and is now a captain with Continental Express.

She was a Sierra flight instructor when she and Thomas met. He was her student. The son of a Danish farmer, Thomas grew up on a 200-acre farm in Denmark that had been in his family for hundreds of years.

When he was 13, his childhood love of flying went airborne after a ride in a restored military aircraft, flown by his father’s friend, a pilot with Scandinavian Airlines.

Thomas’ training and experience as a captain also helped him stay calm as he grappled with his unborn child’s heart problem. “I think we were being very rational about the whole thing,” he said. “We look at the facts, and see what we have to deal with and what we have to work with.”

Term Delivery Was Best Chance for Babies to Survive and Thrive

The facts confronting them, and the Children’s physicians, were simple. Keeping the “plane” containing the fetuses “aloft” as close to term as possible would give both the best chance for healthy outcomes.

Immediately after delivery, the baby with heart block would need a pacemaker. This would give the child an excellent chance for a long, healthy life. But if this fetus developed problems too soon for a healthy delivery, they might be faced with a “lifeboat” problem. What if one fetus died in utero or developed problems requiring premature delivery?

“Do you sacrifice one for the other?” asked Kristine. “We did have to rehearse that in our heads. Losing Katja compounded it. Or maybe it made it less of a shock because we’d been through it before.”

At each prenatal visit, Dr. Rosenfeld carefully examined the size of the heart-block fetus’ heart. If it started to fail, it would first begin dilating. Fluid might collect around the heart (fetal hydrops) or specific heart pulsations might give a clue as to what to expect.

“With one healthy fetus and one not, it’s a constant balancing act,” said Dr. Rosenfeld. “We want the healthy baby to go as close to term as possible, but at the same time we want to be sure we give the baby in heart block the very best opportunity to do well.”

He and the rest of Children’s fetal cardiac service have plenty of experience. They do about 700 fetal echoes a year and install 20 to 25 pacemakers a year. By volume, Children’s Cardiology department has the largest pediatric pacemaker clinic in the Bay Area.

This year alone they’re following more than 150 kids kept alive by pacemakers Children’s clinicians have implanted. Eighty percent or more are placed in newborns. In 14 years they’ve never had a single complication in any pacemaker surgery.

Because the Rasmussen fetuses remained stable, they were delivered at 36 weeks gestation. Cameron, the healthier brother, was born first, followed by Oliver, with his heart still beating in the low 50s. Three Children’s physicians were present to care for Oliver: cardiologist Greg Kurio, MD, and neonatologists Thomas Eusterbrock, MD, and Nick Mickas, MD.

Unanswered Questions for Neonatologist and Parents

Dr. Eusterbrock had felt some anxiety over this moment because he’d also been part of the team caring for the Rasmussens’ first child.

Katja spent two weeks in the Neonatal Intensive Care Unit at Children’s before she passed away. When he learned Kristine was pregnant again Dr. Eusterbrock spent hours studying the literature, trying to understand whether Katja’s death was connected to Oliver’s heart problem.

Kristine had a fever when she delivered Katja. The infant had trouble breathing and was placed on a ventilator. It was thought she was having seizures, and at 1 week of age, she was transferred to Children’s.

She continued having frequent seizures, which didn’t respond to medication and was diagnosed with early myoclonic encepelopathy, a rare disorder with a poor prognosis.

“It was very sad, and also quite frustrating that we couldn’t pinpoint the cause,” said Dr. Eusterbrock. “If Kristine were to get pregnant again, is this potentially something that could happen again? If you have a clear-cut underlying etiology, then you have a better chance to predict the future, and we didn’t have that.”

Concerned about meeting Kristine before her delivery, when she greeted him with a smile and a hug, he was reassured. He also knew that he and his team had thought through every possible scenario.

Oliver Gets a Pacemaker

Dr. Eusterbrock’s presence also helped Kristine. “I was just thrilled that he was going to be there,” said Kristine. “But it was bittersweet. Even though Katja’s passing had been a sad event, you can’t imagine how relieved I was to see Dr. Eusterbrock. I thought, ‘Oliver is going to be well taken care of here.’”

Drs. Eusterbrock and Kurio used external electrodes to pace Oliver’s heart as soon as he was born. But aside from his slow heart rate, the infant was healthy. He was ready for his ambulance ride to Children’s with Dr. Kurio.

Once they arrived at Children’s, the pacemaker team began its work.

Oliver weighed only 2.4 kilos. This led Kishor Avasarala, MD—a Children’s cardiologist specializing in electrophysiology—to choose a very small pacemaker.

Dr. Avasarala’s job includes deciding the size and kind of pacemaker device, the best spot for placement and where to place the leads.

The one he chose for Oliver had only one cardiac lead, meaning the upper and lower chambers of the heart won’t be synchronized until the pacemaker is upgraded in four years.

But the one he picked does contain several advanced capabilities.

• This pacemaker can be fine-tuned from outside Oliver’s body, using a magnet and computer.
• It can regulate heart rate in response to Oliver’s activity level, via a built-in motion sensor, according to limits set by Dr. Avasarala.
• It gathers moment-by-moment data that can be sent to Dr. Avasarala over a phone line using an external wireless device: a form of telemedicine only a year old.
• The lead is coated with drugs to minimize scar formation.
• Its battery lasts up to five years.

Cardiothoracic Surgeon Performs Delicate Pacemaker Procedured

After talking with Dr. Avasarala, Children’s cardiothoracic surgeon Olaf Reinhartz, MD, sewed the button lead directly to the heart’s lower chamber; the other lead went to the pacemaker, placed in the belly wall.

“It’s spectacular that Dr. Reinhartz was able to squeeze the pacemaker into a pocket within the layers of the thin abdominal wall and put the other end on top of a beating heart without poking a hole in it,” said Dr. Avasarala.

Because the pacemaker was small, Dr. Reinhartz only needed to open the lower chest to have room to insert the device. “It took maybe an hour or so,” said Dr. Reinhartz. “It went very well; the main technical issue was sewing the lead on so it has good contact with the heart.”

In about four years, Dr. Avasarala will upgrade Oliver’s pacemaker to a two-lead system, allowing synchronization of the ventricular and atrial chambers. Eventually, when he’s 10 or 11, Oliver will receive a transvenous pacemaker, eliminating the need to sew leads to the heart.

A few days later, the Rasmussens were all at home, resting. The fetal medicine team at Children’s had brought the twins’ plane in for a smooth landing.

“This is an example of how many specialties are involved in one family’s care,” said Dr. Eusterbrock. “A lot of people helped make the right decision in this twin situation. I think that cooperation was really great. There were no surprises for us. We were prepared for all kinds of scenarios. We updated our plans almost on a daily basis. Everybody was prepared for Mr. Rasmussen to arrive.”

The Rasmussens have been pleased with Children’s teamwork and care of all their children, even after losing Katja. Soon after her passing they began making donations to Children’s, to fund neurological research, hoping to help infants born with problems similar to their late daughter’s.

“We knew we wanted to give to Children’s for their research efforts, because they helped us,” said Kristine. “It’s a great organization, it does so much. You think it’s just a hospital, but it’s not, it’s so much more.”

Next spring, when the Rasmussens celebrate their twins’ birthdays, perhaps they’ll all share birthday cake in their backyard, beside the pink roses planted for Katja.

“What’s hard is that the boys look so much like Katja,” said Kristine. “It’s the bittersweet again. You’re so happy they’re here and so sad that she’s gone. They’ll never know her or grow up with her. But I’ll make sure they see her pictures and celebrate her birthday.”

This story, written and photographed by senior writer Tom Levy, was first published in the September 2009 issue of Children’s Hospital’s PEDSnews magazine.