A More Precise Way to Look at Heart Problems

Written by Stephanie Stemmler

Advanced, four-dimensional (4D) MRI imaging technology at SSM Health Cardinal Glennon Children’s Hospital enables heart specialists to detect subtle cardiac problems not visible in more traditional imaging scans.

Bianca and Terrence Williams with their son, TJ, in Forest Park

“Our son had developed a cough that just wouldn’t go away,” recalls Terrence Williams. “At first, our doctor said he didn’t think anything was seriously wrong, but after the cough got worse over a few weeks, we decided to take TJ to the hospital.”

There, X-rays of TJ’s lungs also caught an image of the 4-year-old’s heart. That image made doctors stop and look closer; the right side of TJ’s heart was double the size of the left side. “We were just stunned,” says Bianca Williams. “I mean, we brought him in for a respiratory illness and they found something a lot more serious.”

In addition to the unusually large heart, doctors also listened to TJ’s heartbeat with a stethoscope and heard a slight murmur, which is an abnormal sound of blood flowing through the heart. The Williamses were referred for further evaluation to SLUCare Physician Group pediatric interventional cardiologist Ugonna Nwankwo, MD, at SSM Health Cardinal Glennon Children’s Hospital, where advanced 4D imaging technology diagnosed a rare combination of heart defects.

4D IMAGING

The advanced technology is known as 4D flow cardiac magnetic resonance imaging (4D Flow MRI). Whereas X-rays show images in two dimensions, and CT and MRI scans capture three-dimensional images, 4D Flow MRI takes that one step further.

“This technology not only shows us images of the heart threedimensionally, but it also captures a four-dimensional movie of the heart beating and blood moving through the heart and blood vessels,” says SLUCare Physician Group cardiologist Wilson King, MD, a cardiac imaging specialist.

In other words, 4D Flow MRI showcases movement — how the heart moves, how and where blood flows through the heart and heart valves, which direction it flows and at what speed. It can provide colorful, detailed images that heart specialists can use to diagnose several cardiac problems, including:

• Aneurysms (weak spots in the aorta or heart)

• Cardiomyopathy (thickened, weakened heart tissue)

• Congenital heart defects

• Heart valve problems or diseases

• Tumors in the heart

It was simply serendipity that Terrence and Bianca Williams discovered that their son, TJ, had two unusual heart defects.

The technology has been around for almost a decade, but in recent years advances in image reconstruction, cloud computing and artificial intelligence have enabled it to be implemented clinically. In TJ’s case, he was referred to Dr. Nwankwo and underwent a repeat X-ray and an echocardiogram while at SSM Health Cardinal Glennon. The tests confirmed an enlarged side of the heart as well as a heart valve abnormality. “What we needed was a more detailed analysis to pinpoint the reason and the extent of TJ’s problems,” says Dr. Nwankwo. He turned to Dr. King and SLUCare Physician Group cardiologist and imaging specialist Laura Schoeneberg, MD, who used 4D Flow MRI to track blood flow through TJ’s heart.

The Williams family is all smiles after TJ’s heart surgery

“Because we could see and track blood flow and velocity during the procedure, we were able to identify all the abnormalities in TJ’s heart in a single dataset,” Dr. Schoeneberg says. “They included a narrowed aorta, a heart valve defect, abnormal pulmonary veins and another birth defect that created a hole in the upper chambers of his heart.”

The abnormal pulmonary veins and the hole caused some of TJ’s blood to flow in the wrong direction. The result was that too much oxygenated blood was being pumped back through the lungs instead of to the body. Over time, the problem can cause high pressure in the lungs.

“This is a rare combination of heart defects in a child,” notes Charles Huddleston, MD, a SLUCare Physician Group cardiothoracic surgeon. “I could find only one other report in the literature of a patient with something similar. It took the 4D Flow MRI imaging to detect these anatomic and flow problems in his heart and we used this information for pre-surgical planning.”

Dr. Huddleston also used SSM Health Cardinal Glennon’s 3D Printing Center of Excellence to create a highly accurate plastic 3D model of TJ’s heart, which enabled surgeons to see the degree and angle of surgical reconstruction necessary for the aorta and heart valve. The model also allowed him to closely study the relationship of the abnormal pulmonary veins to the atrial septal defect (hole in the heart).

3D models of TJ’s heart were used for pre-surgical planning

Dr. Huddleston reviewed several treatment options with the family. Bianca and Terrence discussed and prayed about TJ and made the decision for their son to undergo open heart surgery. “It was so much to process when we heard about all these things with TJ’s heart,” said Bianca Williams. “But Dr. Huddleston made us feel comfortable with our decision and the risks involved.”

On the day of surgery, in April 2022, Terrence, Bianca and their pastor gathered to pray just before TJ was taken into surgery. Quietly, by their side, Dr. Huddleston and a nurse stepped up to join them for prayers. “We locked hands together right there; it was so moving for us and I knew then that God was in the midst of this whole thing,” recalls Terrence Williams.

Bianca Williams adds, “It was such a spiritual moment and we needed that so that our child, our whole family, could get through this.”

A visit from Duo Dog Thor cheered up Bianca and TJ while he was recovering in the hospital

TJ’s recovery after surgery was nothing short of amazing. Initially told that he might be in the hospital for seven or more days, TJ was off pain medications and a breathing tube by the third day. On the fourth day, he went home.

“We locked hands together right there. It was so moving for us and I knew then that God was in the midst of this whole thing.”

Today, TJ is back to being a happy, active child. As part of his medical evaluations, he underwent genetic testing to see if there was an underlying reason for his heart problems. In addition to his rare combination of heart defects, TJ has another rare congenital condition called Kabuki syndrome, which has more recently been linked to congenital heart defects.

A happy TJ runs toward his parents in Forest Park

“TJ’s particular heart defects would have been difficult to fully diagnose with traditional imaging,” notes Dr. Nwankwo. “4D Flow MRI gave us a better, more detailed picture of blood flow and anatomy, which enhanced our ability to diagnose and then treat all of his heart problems.”

On a recent day in Forest Park, the Williams family takes joy in the simple pleasure of walking and running around together. “No one would ever know our son had open heart surgery unless he lifted up his shirt to show off his scar,” says Bianca Williams. “He’s so active with everything and this is a testament to the doctors and the technologies they used to help him get back on his feet.”