Because Mili’s heart defect was diagnosed during pregnancy, the team at Children’s was able to prepare a plan of care for Mili’s family. Andrea Jedwab and Adrian Breitfeld are the proud parents of a lively baby girl who fills their life with joy. But little Mili Breitfeld, who joined her then- 20-month-old brother Liam in May 2012, is very lucky her mom believed in getting good prenatal care.
Andrea and Adrian were preparing to move to the Bay Area from Los Angeles when her obstetrician’s office called to say there was something unusual about the prenatal ultrasound they had performed early in Andrea’s pregnancy. “They wanted me to come in for another ultrasound in L.A., but we were moving to Emeryville that following weekend,” she recalls. “So as soon as we could, we found a local obstetrician at Alta Bates, who ordered the follow-up ultrasound.”
Her new doctor also saw something that “wasn’t right.” He referred Andrea to the Pediatric Cardiology Division at UCSF Benioff Children's Hospital Oakland, where they performed a fetal echocardiogram when she was 18 weeks pregnant.
“A fetal echocardiogram is a type of ultrasound that provides a moving picture of the developing baby’s heart,” says Children’s Chief of Pediatric Cardiology Howard Rosenfeld, MD, an expert in fetal echocardiography. “In this case, the ‘echo’ showed a serious defect called ‘transposition of the great arteries,’ in which the two large blood vessels that carry the blood out of the pumping chambers of the heart—the pulmonary artery and the aorta—were ‘transposed,’ or switched, during the development of the heart.”
Early Detection and Correction Critical to Survival
Most cases of transposition of the great arteries are diagnosed in the first hours or days of life because the baby has low oxygen levels and may appear “blue” rather than a normal, healthy color. Babies with this defect also breathe rapidly, and their hearts work harder than normal to try to get more oxygen to the body. If the defect is not corrected, there is a 50-percent chance the baby will die within the first month of life and a 90-percent chance of death within the first year. Any delay in diagnosis and treatment may result in serious problems for the child, including brain damage or damage to other organs such as the liver and kidneys.
Children’s cardiologists and cardiac surgeons are an integral part of the Fetal Medicine Center at Children’s. This center cares for the fetus before birth and provides rapid diagnosis and management of fetal abnormalities by a team of pediatric specialists. Because Mili’s heart defect was diagnosed during pregnancy, the team at Children’s was able to prepare a plan of care for Mili’s family.
“It’s a great advantage to have a prenatal diagnosis for conditions like Mili’s,” says Dr. Olaf Reinhartz. “This type of surgery could not be performed in a hospital without pediatric cardiac surgery specialists.”
Children’s Fetal Medicine Center has had an association with Alta Bates Hospital in Berkeley and John Muir Hospital in Walnut Creek for many years. The program is expanding. “Our goal is to provide a fetal medicine program that responds to the needs of families, obstetricians, and perinatologists throughout the East and North Bay,” says Dr. Rosenfeld. “By working closely with families and care providers in complex pregnancies, we can safeguard fetal health while honoring parents’ desires to deliver their babies close to home. We also can provide families and their physicians with appropriate information so they can prepare themselves for what to expect.”
Andrea and Adrian were frightened by the diagnosis of their baby’s heart defect, as any parent would be, but they did appreciate the time they had to prepare.
“I was scared to death,” Adrian admits. “Early on, waiting for the test results was difficult, not knowing what might be wrong. Being surprised by Mili’s condition at birth would have been even more traumatic, though. Having the advance notice helped a lot; we were well prepared.”
Andrea agrees: “It was definitely scary to get the diagnosis and learn something was wrong with our baby’s heart. Then we had our first follow-up appointment with Dr. Rosenfeld, who explained everything very carefully and clearly. I was grateful to learn about the defect early in my pregnancy so I could be prepared for what would happen. The people at Children’s and my obstetrician at Alta Bates were prepared and knew what to do, too. That wouldn’t have happened if we hadn’t known about the heart defect ahead of time.”
Getting Ready for Mili’s Birth and Surgery
Andrea kept up her regular prenatal exams with her obstetrician and additional appointments with Dr. Rosenfeld, with frequent echocardiograms to monitor the baby’s heart. Dr. Rosenfeld coordinated the care plan with Medical Director of Cardiology Hitu Patel, MD, and Chief of Pediatric Cardiac Surgery Olaf Reinhartz, MD. Dr. Patel, who specializes in cardiac catheterization procedures, would be responsible for improving and stabilizing the oxygen flow to the baby’s body. Dr. Reinhartz would perform the surgery to correct the transposed arteries.
The Children’s medical team was notified as soon as Andrea went into labor, and baby Mili was transported from Alta Bates to Children’s within hours of her birth. Adrian and his brother went to Children’s to be with Mili while Andrea recovered overnight at Alta Bates.
Going from Blue to Pink
The medical team got to work right away raising Mili’s blood-oxygen level, which was a dangerously low 60 percent. The doctors used medication to help keep open a small blood vessel near the heart that connects the pulmonary artery and the aorta, allowing some mixing of oxygen-rich blood with oxygen-poor blood. Then Dr. Patel performed a heart catheterization procedure to create an opening between the upper chambers of the heart to allow more blood to mix. Dr. Patel notes that fetal diagnosis of a condition like Mili’s helps take the guesswork out of what procedures will be necessary. “The trick is to stabilize the baby quickly,” he says. “Fortunately, the technology for cardiac catheterization just keeps getting better and better, and the equipment is now designed just for babies. As soon as Mili’s catheterization was done, her blood-oxygen level rose to 85 percent, which was a very good improvement.”
Mili’s father noticed the difference right away. “The attention and care Mili received from the doctors at Children’s was evident from the get-go,” Adrian says. “Dr. Patel’s optimism helped me relax. I was concerned because when she went in to the catheterization lab, she was blue. When she came out about an hour later, she was pink!”
That first night, Mili needed some assistance with her breathing, and for the following two nights, the staff at Children’s kept a close watch to make sure she was ready for corrective surgery three days after her birth.
A “Fantastic” Surgery
Correcting transposition of the great arteries involves a complex surgical procedure called an “arterial switch.” Mili’s surgeon, Dr. Reinhartz, describes the arterial switch procedure as a “fantastic surgery” that illustrates the advances made in pediatric cardiac surgery.
“Basically, we take the aorta and the pulmonary artery off the places where they connect to the heart and move them to the proper position,” says Dr. Reinhartz. “We also have to switch the smaller coronary arteries that supply blood to the heart muscle itself. This is technically the trickiest part of the surgery because the coronary arteries are much smaller blood vessels that require microsurgical techniques that were not available until about 20 years ago.”
After surgery, Mili’s blood-oxygen level went up to 100 percent right away. She spent only 12 days in the hospital after surgery, and Dr. Reinhartz notes it is very unlikely she will need another surgery. “It’s a great advantage to have a prenatal diagnosis for conditions like Mili’s, and to schedule the birth near the hospital where surgery can be performed,” he adds. “This type of surgery could not be performed in a hospital without pediatric cardiac surgery specialists. Having a prenatal diagnosis can make a big difference in the baby’s chances of survival.”
During the five-hour surgery, Mili’s mom and dad paced the floor, but they note that they received great care, too. “Everyone was so attentive and caring,” Andrea explains. “They gave us a pager, and people came out every hour or so to give us updates. We were very happy with all the care we received at Children’s, and we have absolute confidence in the doctors. We were in good hands, and it was wonderful to be at a hospital close to home.”
Mili’s family recently moved again, this time to a larger home in Pleasant Hill, where they will still be close to the expert care they found at Children’s in Oakland. Mili’s cardiologist, Dr. Rosenfeld, who will continue to follow her case until she reaches age 21, expects her to lead a completely normal life.
“We’ve been blessed that Mili’s surgery was successful and that she recovered so quickly,” says father Adrian. “It’s wonderful to be able to boast that Mili is a beautiful, healthy baby.”
FETAL MRI: A VALUABLE TOOL FOR DETECTING FETAL ANOMALIES
UCSF Benioff Children's Hospital Oakland currently is the only hospital in the East Bay to offer fetal MRI (magnetic resonance imaging) as a complementary diagnostic imaging tool when suspected fetal anomalies are not well assessed by ultrasound. A proven, well-established diagnostic imaging technique, MRI uses a strong magnetic field and radio waves to obtain pictures of the inside of the body without using radiation.
There are numerous conditions for which fetal MRI imaging may be indicated, according to Children’s Director of Neonatology Art D’Harlingue, MD. Dr. D’Harlingue heads Children’s Fetal Medicine Program which provides rapid diagnosis of abnormalities and cares for the fetus before birth and management of abnormalities after birth by a team of pediatric specialists.
“An obstetrician or perinatologist may detect a potential birth defect with ultrasound, but there may not be enough detail to differentiate between conditions that may look alike on a sonogram,” says Dr. D’Harlingue.
“Current data show that the magnetic field and the radio waves used in fetal MRIs are safe for the mother and the unborn baby,” says Pinar Karakas, MD, a board-certified pediatric radiologist at Children’s Hospital who has substantial experience in analyzing fetal MRIs. “Fetal MRIs have been used for more than 15 years without a reported negative effect on the imaged fetuses. Pregnant patients can be accepted to undergo MRI scans at any stage of pregnancy but are preferably performed in the second trimester after 20 weeks of gestation.”
FETAL MEDICINE TEAM AT CHILDREN’S OAKLAND
The Fetal Medicine team at Children’s Hospital Oakland serves as a single point of contact for expectant parents with prenatally diagnosed conditions.
Our team of specialists works with obstetricians, perinatologists, and pediatricians throughout Northern California to make sure infants with prenatally diagnosed conditions get the best possible care during pregnancy, delivery, the newborn period, and beyond. By coordinating prenatal consultation and multi-disciplinary planning, we work to ensure that infants get the care they need, and parents know what to expect.
CONSULTATION & REFERRAL
Erin Corbett, RN, PNP
Fetal Medicine Coordinator