Advanced Imaging Technology Detects Rare Congenital Heart Condition

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

“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, an x-ray of TJ’s lungs also caught an image of the 4-year-old’s heart. Doctors discovered that the right side of TJ’s heart was double the size of the left side. “We were stunned,” says Bianca Williams. “We brought him in for a respiratory illness and they found something a lot more serious.”

The Williams’ were referred for further evaluation to SSM Health Cardinal Glennon Children’s Hospital where pediatric interventional cardiologists and heart imaging specialists diagnosed a rare combination of congenital heart defects through the use of advanced four-dimensional flow magnetic resonance imaging (4D Flow MRI) technology.

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 shows us images of the heart three-dimensionally and captures a four-dimensional movie of the heart beating and blood moving through the heart and blood vessels,” says cardiologist Wilson King, MD.

4D Flow MRI, then, showcases movement — how the heart moves, how and where blood flows through the heart and heart valves, which direction blood flows, and at what speed. It can provide colorful, detailed images that heart specialists 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

“Because we could see and track blood flow and velocity through imaging, we were able to identify all the abnormalities in TJ’s heart,” says cardiologist and imaging specialist Laura Schoeneberg, MD. “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.”

“This is a rare combination of heart defects in a child,” notes cardiothoracic surgeon Charles Huddleston, MD. “I could find only one other report in the literature of a patient with something similar. It took the 4D Flow MRI technology to detect these anatomic and flow problems in his heart.

“It was so much to process when we heard about all these things with TJ’s heart,” recalls Bianca Williams. “But Dr. Huddleston made us feel comfortable with our decision to go with open heart surgery to fix the problems.”

Pre-surgical planning included the creation of a highly accurate 3D model of TJ’s heart, which enabled surgeons to physically see the degree and angle of surgical reconstruction necessary. The model was created in SSM Health Cardinal Glennon’s 3D Printing Center of Excellence.

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. “It was so moving for us,” says Terrence Williams. “I knew then that God was in the midst of this whole thing,” Bianca Williams agreed, saying “It was such a spiritual moment and we needed that so that our child, our whole family, could get through this.”

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

Today, TJ is back to being a happy, active child. “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, and this a testament to the doctors and the technologies they used to help him get back on his feet.”