Cutting-edge heart care begins before birth

By building one of the nation’s premier fetal heart programs, Children’s Wisconsin’s renowned cardiology care spans from the fetal stage through adulthood

It’s been nearly three decades since Children’s Wisconsin hired Michele Frommelt, MD, to start the hospital’s first Fetal Heart Program. Back then, fetal imaging meant relatively simple ultrasounds with limited imaging of the heart, and fetal cardiology was a new specialty.¹
Today, fetal cardiology is considered one of the new frontiers within pediatric cardiology and is one of the largest programs at Children’s. Every year, the program’s four fetal cardiologists within the nationally-recognized Herma Heart Institute see more than 500 women and conduct more than 700 fetal echocardiograms.
Building one of the premier fetal heart programs in the country wasn’t easy, particularly in an era in which obstetricians referred all such patients to maternal fetal medicine (MFM) specialists. So Dr. Frommelt, the program director, took her message on the road. “For years, I would do my regular day job and then on my way home stop at the MFMs’ offices to review ultrasounds with them,” she said. The result was a strong community partnership that ensures the best care for every patient.

Today, all fetal cardiac imaging is performed in the new Fetal Concerns Center, located right across the hall from the Herma Heart Institute. The close proximity of the two is not coincidental. Before, all fetal cardiac imaging was performed in the Herma Heart Institute, and the MFMs were located at Froedtert Hospital. This meant families often had to visit both places — about a 10-minute walk apart — in a single appointment. Moving the two just 15 steps apart not only improves the patient experience, but it fosters better communication and collaboration between clinicians.

Finding a problem

Congenital heart defects are the most common birth defect in the US², affecting an estimated 1 percent of babies (40,000) born every year in the US. The Fetal Heart Program was designed for and is dedicated to these kinds of complications. “In general, a referral to the Fetal Heart Program is made when there is a risk factor identified in the mother, in the fetus, or in the family,” Dr. Frommelt said.

For instance, she said, mothers with diabetes mellitus have a fivefold greater risk of delivering a baby with congenital heart disease compared to women without the disease. Fetal cardiac risk factors include chromosomal abnormalities, such as Down syndrome, as well as “suspicious” views, such as an abnormal four-chamber view on a routine anatomy scan of the heart. A referral for an abnormal four-chamber view results in a complex congenital heart disease diagnosis in more than half of patients, she said. Familial risk factors include heritable disorders of the heart, such as a bicuspid aortic valve or hypertrophic cardiomyopathy.

Another indication for a fetal cardiology referral is the presence of a fetal arrhythmia, which occurs in about two percent of pregnancies. While many are benign and resolve on their own, others can lead to significant morbidity or mortality. It is, in fact, thought to be a primary cause of sudden, unexplained fetal death, sometimes called stillbirth.³

Fetal magnetocardiography at Children’s Wisconsin

The most common diagnostic approach to assess the type and severity of fetal arrhythmia is fetal echocardiography. However, its utility in arrhythmias is limited because it relies on wall motion and blood flow rather than the heart’s electrical activity, which a surface EKG provides.

The fetus is in a little “insulated cocoon” from which electrical signals don’t escape, but luckily, magnetic signals can and do escape. The Fetal Heart Program at Children’s Wisconsin is one of the only programs in the country — and the world — to offer fetal magnetocardiography (fMCG). Its noninvasive, passive recording technique functions much like an EKG, only instead of recording electrical signals of the heart, it records the magnetic fields the heart generates. The device is safe, because it does not produce magnetism (it’s not an MRI), rather it captures naturally-occurring magnetic signals from the fetus. This enables doctors to identify the type of arrhythmia and its underlying mechanism, allowing for better treatment.

One of the pioneers of fMCG, Janette F. Strasburger, MD, a professor of Pediatric Cardiology at the Medical College of Wisconsin, is part of the Children’s team. Dr. Strasburger and the Medical College received several National Institutes of Health grants to conduct research in this area, most recently a $2.4 million R01 grant to assess the role of “hidden” fetal arrhythmia in fetal death. To ensure this new diagnostic tool is available to all moms as needed, the hospital has developed billing codes for fMCG and is partnering with national organizations and insurance companies to enable insurance coverage.

For more information on the NIH studies on fetal echocardiograph or fetal magnetocardiography, visit ClinicalTrials.gov. Dr. Strasburger co-wrote the section on fetal arrhythmias for the AHA Scientific Statement on Fetal Diagnosis and Treatment published in 2014.⁴

Seamless Transition

Diagnosis of a congenital heart defect is just the first step in the care continuum at Children’s. Prenatal counseling begins as soon as a problem is identified.

“Much of what I do is help families understand what’s going on with their baby,” Dr. Frommelt said. “Studies find that this kind of counseling helps families handle the psychological stress of a prenatal diagnosis and can even improve long-term outcomes.”

From the first appointment to delivery, families interact with one of two fetal heart program nurses who coordinate their care and provide a consistent resource throughout the pregnancy and beyond. “It’s really nice for families to know that they can always contact one person who will know everything about them, their baby and their upcoming appointments,” Dr. Frommelt said.

A unique feature of Children’s Wisconsin is that it is one of the few Children’s Wisconsins in the country co-located with a high-risk delivery unit. This means that babies with a prenatal diagnosis of congenital heart disease can be delivered in the same location in which they will receive follow-up specialty care, significantly reducing the risks associated with transport to and from another facility. “More importantly, the baby has immediate access to the multidisciplinary team taking care of them, and the mother is in close proximity to the baby at all times,” Dr. Frommelt said. This promotes maternal-infant bonding while significantly reducing stress on the family.

Post-delivery interventions are performed at the Herma Heart Institute, which has one of the highest ratings for congenital heart surgery from the Society for Thoracic Surgeons. U.S. News and World Report also ranks it among the top pediatric heart programs in the country. In addition, the Institute has the best published survival rates worldwide for hypoplastic left heart syndrome.

The continuum of care extends throughout the patient’s life, with access to one of the top adult congenital heart disease (ACHD) programs in the country. As an integral part of the Herma Heart Institute, the ACHD Program works together with cardiovascular experts to ensure a smooth transition. The program is accredited as a Center of Comprehensive Care by the Adult Congenital Heart Association.

Planning for Delivery

Planning for the delivery begins by determining the severity of the fetal heart defect.

“We developed an algorithm that assigns levels of concern to the fetus so that the multidisciplinary team involved with the delivery is aware of the post-delivery requirements,” Dr. Frommelt said. For example, a fetus with a benign arrhythmia is assigned a level one, which means the baby can go to the regular nursery after birth with orders for an EKG before discharge. Level five fetuses have severe congenital heart disease that requires an intricately planned delivery, often with the need for immediate post-delivery surgery or intervention. The co-located birth center proves to be of immense value for these level five fetuses, as the operating room is only a few floors away from delivery.

“These mothers often require a caesarian section so that the needed team of specialists, including pediatric cardiology surgeons, intensivists and neonatologists, can be on site at the delivery for possible intervention,” Dr. Frommelt said.

So why refer to the fetal heart program at Children’s Wisconsin?

“Our multidisciplinary team is dedicated to improving the lives of our patients with congenital heart disease through innovative research and patient care that spans the continuum of care from fetal life to adulthood,” Dr. Frommelt said. “This, in turn, has led to our program producing some of the best outcomes in the country.”

References

1 Medrano-Lopez C, Fouron J. Fetal Cardiology. the Frontier of Pediatric Cardiovascular Medicine. Rev Esp Cardiol. 2012;65:700-4.
2 Centers for Disease Control and Prevention. Number of U.S. Babies Born with CHDs. Available at: https://www.cdc.gov/ncbddd/heartdefects/data.html
3 Wacker-Guissmann A, Wakai RT, Strasburger JF. Importance of Fetal Arrhythmias to the Neonatologist and Pediatrician. Neoreviews. 2016 Oct; 17(10): e568–e578.
4 Donofrio MT, Moon-Grady AJ, Hornberger LK, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014;129(21):2183-2242