Two surgical Teams Save Pregnant Mom With Marfan Syndrome

Beth O’Connor hoped the sharp, searing pain that started in her chest and shot down to her belly was labor pangs and not her aortic artery coming apart. The pain brought Beth, who was almost 39 weeks pregnant and scheduled for a cesarean in two days, to her knees in a Waldo parking lot.

She called her husband, Glen.

“Stay there,” he said. “I’m on my way.” He feared if his wife lost consciousness, paramedics might assume it was labor or a heart attack. A delay in identifying an aortic dissection could be fatal to two lives.

The O’Connors were quick studies on aortic dissections after learning Beth has Marfan syndrome, a life-threatening genetic disorder that can cause an aortic dissection. That’s a tear in the inner layer of the aorta, the large blood vessel branching off the heart.

Their crash course on Marfan began a few weeks earlier when Saint Luke’s pediatrician Nastasha Burgert, MD, was doing a routine checkup on Beth’s seven-year-old daughter. The girl’s 5-foot 2-inch height, flexible joints, and slightly protruding chest prompted Dr. Burgert to suggest an evaluation for Marfan syndrome. Beth was tested as well.

During the evaluation, an echocardiogram showed Beth’s aorta was dilated enough to make labor dangerous. Ibrahim M. Saeed, MD, a cardiologist with Saint Luke’s Mid America Heart Institute, recommended a cesarean on Dec. 9, 2013, the day her baby would be 39 weeks.

The right choice

As Glen drove Beth to Saint Luke’s Hospital, they discussed whether to go to the Emergency Department or the Maternity Center. They chose the latter.

“We were both in denial, hoping this was labor,” Beth said.

When Glen explained his wife had Marfan and chest pain, a Maternity nurse wheeled Beth to the Emergency Department.

A CT scan revealed that Beth’s aorta had separated from her carotid artery in her neck all the way down to her legs, and her coronary valve had disintegrated. Repairing the lower portion below her heart could wait, but the ascending aorta could rupture and hemorrhage at any moment.

“Two lives were hanging in the balance—the mother and her unborn child,” said Sanjeev Aggarwal, MD, a cardiothoracic surgeon who specializes in treating Marfan.

Glen said goodbye to Beth as she was taken into surgery at 9:30 p.m. “It would be the longest night of my life,” he said.

A full house

For a typical heart valve repair, a cardiovascular surgical team would include seven or eight. But Beth and her unborn child needed an obstetric and neonatal team, as well. Her surgical team totaled 27.

“It testifies to the depth of talent at Saint Luke’s Hospital to be able to coordinate and make this happen,” Dr. Aggarwal said.

To avoid subjecting her baby to the blood thinners, body cooling, and medications necessary to put Beth on an ECMO machine, the cesarean came first. ECMO, or extracorporeal membrane oxygenation, keeps blood flowing and the oxygen circulating by acting as artificial heart and lungs.

As the cardiac team saw OB-GYN Douglas R. Perryman, MD, deliver the 7.3-pound baby girl at 10:22 p.m., there was profound quiet.

“Once we saw the healthy, beautiful baby, we all knew we would work as hard as humanly possible to make sure her mother would live to care for her,” said Dr. Aggarwal. “Repairing an aortic dissection is one of the most complex operations you can perform on the human body,” he said. “And Beth’s was one of the most extensive repairs I’ve ever done.”

During the 13-hour surgery, the cardiac team had to open her chest, stop her heart, and put her on ECMO before Dr. Aggarwal removed the defective portions, replaced the aortic valve, and repaired three parts of the aorta.

Better together

The maternity and heart teams accommodated the new family, with mother and baby on opposite sides of the hospital. For example, nurses understood how critical mother-newborn bonding is, so they regularly brought the baby from the Nursery to the Cardiovascular Intensive Care Unit, where Beth was recovering.

Even under sedation, Beth would visibly relax when they placed the newborn on her chest, and the baby would do the same—as they enjoyed the first minutes of life together.

One day Glen noticed the nursery was named in memory of Mary Beth Connor and the first friends to hold her were both named Mary Elizabeth. It was fate: When his wife awoke, he suggested Mary Elizabeth as a name for their baby.

Today Beth describes Mary Elizabeth as “a happy, healthy infant who is already crawling and impatiently trying to hoist herself up—as normal as can be.”

What’s much more amazing, so is her mother.