Providing complex care for the tiniest heart patients

In recognition of Congenital Heart Defect Awareness Week, we recently spoke with Dr. Majd Makhoul, director of the Fetal Cardiovascular Program at UK HealthCare Kentucky Children’s Hospital, about how we diagnose and treat babies with fetal heart conditions. Kentucky Children’s Hospital and Cincinnati Children’s Joint Pediatric Heart Program is ranked 15th in the country in pediatric cardiology & heart surgery. Dr. Makhoul told the story of a boy whose heart condition was discovered during an ultrasound. 

Tell us about the baby you recently treated.

Our ultrasound technologists travel to locations throughout Kentucky to perform fetal ultrasounds for patients who don't live near Lexington. The mother in this case received an ultrasound scan at one of our telehealth outreach locations and learned her baby had an enlarged heart.

A fetal echocardiogram (fetal echo) confirmed the baby had cardiomyopathy, a condition that affects the heart muscle. Our Maternal-Fetal Medicine (MFM) team, which treats high-risk pregnancies, and our Fetal Cardiovascular Program team worked together to monitor the baby and mother very closely throughout the pregnancy. A team of pediatric specialists developed a coordinated plan for delivery and treatment.

At 34 weeks, the baby boy was delivered at UK. He was very sick, though. He had several procedures and required a ventilator. The baby slowly started to get better, and after spending seven weeks in the neonatal and pediatric cardiac intensive care units (ICUs), he was able to go home. While he still needs close observation and multiple medications, he is with his family at home now. The family understands he may need a heart transplant in the future, but for the time being, he is growing and doing well.

How do patients get referred to your team?

Typically, mothers see us if they have certain medical conditions or a history that puts them at high risk for fetal heart conditions. This includes mothers who previously had a child with a heart condition or had a heart defect of their own. Patients are also referred to us if something unusual is spotted during their 20-week routine ultrasound. Our highly trained fetal cardiac sonographers perform the fetal echos at UK and other affiliated centers around Kentucky. They are the frontline soldiers and do an amazing job. We can find complex defects as early as 18 weeks in some cases.

How do you diagnose babies with fetal heart conditions?

We primarily use fetal echo. This specialized test uses ultrasound technology, which is safe for the fetus. We also occasionally perform blood work to check for certain proteins in the mother’s blood that can affect the baby. If we’re treating a fetal arrhythmia by giving the mother medications, we need to monitor her with EKGs and blood testing.

How do you treat babies when a fetal heart condition is diagnosed?

In some cases, we will treat the baby in utero by giving the mother certain medications and monitoring carefully. After birth, interventions could include medications, balloons and devices inserted during a visit to the cardiac catheterization laboratory (cath lab), open heart surgery or heart transplant.

We have a large team of pediatric providers trained in a variety of specialties and subspecialties who all work together to determine and carry out the treatment plan for each mother and baby.

What support is available for families? 

This experience can be overwhelming, and we try to make it easier for families. We take the time to talk with each family about the fetal heart condition and plan for delivery and treatment. Families have the chance to tour the facility and meet team members who will take care of their baby before delivery. 

Our fetal nurse navigator stays in touch with families and answers questions. Our licensed clinical social workers help families know what to expect and how to cope. They connect families with resources and others in similar situations. They even help prepare siblings. We also have a fetal coordinator who works with the patients and the referring Maternal-Fetal Medicine team to schedule appointments and arrange transportation.

How does the fetal heart team collaborate with the MFM team?

Even with great technological advances, some heart conditions are harder to diagnose than others. A routine ultrasound can miss up to half of all fetal heart defects, while a focused fetal echo can catch 90 percent of them. We have a great relationship with the MFM team. They will reach out to us if there are any questions that increase the chance of catching those congenital heart defects.

These babies often need specialized pediatric care after birth, and very few centers in Kentucky can provide that level of care. We are the only pediatric cardiac center in Central and Eastern Kentucky. By making the diagnosis before birth, we improve the outcomes of some heart defects with proactive care.

We also train stenographers at 15 hospitals throughout the state. They can perform echocardiograms after birth if there are any questions about the baby’s heart. Sonographers then send digital images to us for review. If there’s a significant issue, the baby gets transferred to UK.

What is the outcome for children with heart conditions?

Many of the children we take care of do great and live normal lives. For a certain percentage who need ongoing support into adulthood, we work with our colleagues on the adult congenital cardiology team to provide care at UK from birth all the way through adulthood.

For more information or to schedule an appointment with Kentucky Children's Hospital providers, call 859-323-5000 or click here to visit our website.