All heart: UCLA docs guide mom with heart condition through birth, operate on newborn

The 33-year-old from of Bakersfield, Calif., suffers from a congenital heart disease called Ebstein’s malformation of the tricuspid valve, and from abnormal pulmonary veins. She has undergone multiple surgeries over her lifetime, including one to repair a hole in her heart, a valve replacement and the implantation of a pacemaker.

Although she has a 13-year-old daughter (and an adopted 3-year-old daughter), doctors felt she would likely never be able to have more children because of her condition.

Then, in early 2011, a year-and-a-half after her last surgery at Ronald Reagan UCLA Medical Center, to replace a tricuspid valve, Cole had become so much stronger and healthier that she unexpectedly became pregnant. Her local doctors advised that the pregnancy would be life-threatening to her and her unborn baby, and they recommended that she terminate the pregnancy.

Instead, she sought out experts at UCLA experienced in the management of high-risk pregnancies and high-risk babies. UCLA is one of a select number of medical centers in the country where high-risk expectant mothers and their unborn children can be cared for by experts in pediatric and adult congenital cardiology, maternal-fetal medicine and congenital cardiothoracic surgery. Infants can be delivered and undergo the most advanced lifesaving pediatric heart surgery within days of their birth without needing to be transferred to another specialty hospital.

"Our goal is to give our patients the hope, confidence and means to live a normal life — including helping a mother have her baby," said Jamil Aboulhosn, an assistant clinical professor of cardiology and co-director of the Ahmanson/UCLA Adult Congenital Heart Disease Center. "Patients born with complex congenital heart disease 60 years ago would likely not have survived into adulthood. Given our medical and surgical advances, many patients are now living long, fulfilled lives."

Mark Sklansky, chief of pediatric cardiology at Mattel Children’s Hospital UCLA , performed a fetal echocardiogram using state-of-the art technology and found that the unborn baby had a serious, life-threatening congenital heart problem called double inlet left ventricle, meaning it would be born with only one pumping chamber. The baby would require advanced, risky open-heart surgery within days of her birth to survive.

"Knowing the baby needed this surgery, we were able to plan ahead," Sklansky said. "Our entire team, including adult cardiology, pediatric cardiology, obstetrics, maternal–fetal medicine, heart surgery, neonatology and nursing all came together to plan each step of the way."

Brian Reemtsen, an assistant professor of cardiothoracic surgery who had performed Cole’s latest tricuspid valve replacement, would operate on the newborn baby too.

"With this complex form of double inlet left ventricle, we essentially need to re-do the heart’s plumbing so that the one chamber can pump efficiently and supply blood to the lungs," Reemtsen said. "It’s a high-risk operation that must be done the first week of life."

"The good outcome for this complicated pregnancy is most gratifying," said Brian J. Koos, a professor of obstetrics and gynecology. "As a subspecialist in maternal–fetal medicine, I am pleased to have been part of the multidisciplinary team that provided state-of-the-art care for Keyota and Faith."

Immediately following the birth, Faith was wheeled to the neonatal intensive care unit, located on the same floor as the delivery area, for monitoring and to await her surgery.

"Parents are comforted in knowing that everything their baby could need is available here, with all the expertise and technology," said Uday Devaskar, a professor of neonatology at Mattel Children’s Hospital UCLA. "There is nowhere else that can offer a higher level of care."

Just five days after birth, Faith underwent the complex four-hour surgery to fix the plumbing in her heart. She will need another, less risky surgery when she is between four and six months old, and then again at 3 years old, to further refine the original surgery. Reemtsen anticipates that Faith will have some physical limitations as she grows older, but his hope is that she will lead a normal life.

"My heart may never be perfect, and her heart is never going to be perfect, but we are alive and we are here," said Cole. "My hope for Faith is that she’ll have a wonderful life, just like her mother."

The UCLA Health System has for more than half a century provided the best in health care and the latest in medical technology and research to the people of Los Angeles and the world. The UCLA Health System is among the most comprehensive and advanced health systems in the world, comprising Ronald Reagan UCLA Medical Center; UCLA Medical Center–Santa Monica; the Resnick Neuropsychiatric Hospital at UCLA; Mattel Children’s Hospital UCLA; and the UCLA Medical Group, with its wide-reaching system of primary care and specialty care offices throughout the region.