Building a Fontan Program to Help Patients Thrive Through Childhood – and Beyond

Fontan surgery has significantly improved the lives of children with single ventricle defects, enabling many of them to lead relatively healthy, active childhoods. But the risk of serious complications rises as these patients near adolescence and young adulthood. That’s why The Heart Center at Children’s Health℠, Children’s Medical Center Dallas created one of the nation’s first programs dedicated to helping Fontan patients stay as healthy as possible for as long as possible.

Our multidisciplinary program brings together providers with expertise in every key organ system, with the goal of preventing, managing and treating complications unique to Fontan patients. This program includes, to our knowledge, North America’s second pediatric cardiac lymphatic occlusion program, which aims to mitigate issues such as plastic bronchitis and recurrent pleural effusions.

“There are at least 15,000 Fontan patients in the U.S. today but fewer than 4,000 hearts are available for transplant each year,” says Suren Reddy, M.D., Pediatric Cardiologist at Children’s Health and Associate Professor at UT Southwestern. “We created our Fontan-Single Ventricle Program to help kids keep their hearts for as long as they can, and our large patient population gives us the means to find ways to further improve the standard of care.”

Partnering with Primary Cardiologists to help Fontan Patients

The moment the Fontan surgical suture lines are completed, the liver, intestines, airway, bronchial lymphatic collaterals and the rest of the systemic venous pathway are subject to significantly elevated pressures. This triggers a chain reaction that can lead to an array of serious health issues.

Primary cardiologists are typically charged with monitoring and caring for many of these issues. Our program works hand-in-hand with these physicians to supplement their care with specialized testing, evaluation and treatment.

“There is a constant stream of new data and publications related to Fontan patients,” Dr. Reddy says. “We stay on top of this information so we can add to our expertise and help primary cardiologists ensure their Fontan patients get the latest, most effective care.”  

Preventing Complications from Fontan Procedures

Apart from dedicated cardiologists who specialize in single ventricle pathophysiology, adult congenital heart disease and heart failure/transplant, our Fontan-Single Ventricle clinic includes neurodevelopmental specialists, hepatologists, pulmonologists, social workers, nutritionists, nurse practitioners, physical therapists and other specialists. We have dedicated clinic days where providers’ schedules are aligned to care for Fontan patients, so patients and families can see all the necessary specialists in the same place, on a single day.

“We get referrals from across Texas and from several other states,” explains Mellissa Myers, FNP, “and we try to make families lives easy by enabling them to see their whole treatment team in one visit.”

When a patient is referred, our team starts by evaluating their lungs, liver, kidney and other organ systems, and also conducts a neuropsychological evaluation. Then we develop a care plan that outlines the tests and treatments that could help in the coming years. We deliver that plan to the primary cardiologist and the family, and work together to decide which steps to pursue.

“If a patient is doing well, they typically continue care under their primary cardiologist and return to our clinic every two to three years for multidisciplinary evaluation,” Dr. Reddy says.

In other cases, our comprehensive screening identifies complications early. For example, a patient was recently referred to us for a routine Fontan screening. Their echocardiogram was normal but CT and MRI scans revealed a coarctation of the aorta. We used a catheterization procedure to dilate the aorta and eliminate the obstruction.

“The patient was asymptomatic and would have continued doing fairly well for months or years, and would have then come back to us with significant valve leakages or decreased heart function,” Dr. Reddy says. “Fortunately, our screening protocols enabled us to catch the problem and address it early.”

Building a Lymphatic Occlusion Program

Our team is constantly embracing and developing new pathways and approaches. That’s one reason we built a Lymphatic Occlusion Program to help Fontan patients overcome issues including plastic bronchitis and recurrent pleural effusions. This can require closing the lymphatic collateral vessels and sometimes the entire thoracic duct to prevent lymph from leaking through the duct and invading the lungs.

The lymphatic occlusion program builds on the teamwork already established in our hybrid catheterization/MRI lab. This lab was established in 2017 and was the first of its kind in Texas and is one of only a few in the country. 

“Lymphatic occlusion procedures require seamless collaboration, so it was extremely helpful that we already had an established flow between the imaging team and the cath lab,” Dr. Reddy says.

During lymphatic occlusion procedures, our MRI team lights up the thoracic duct and the surrounding lymphatic communications. This enables Dr. Reddy and Sheena Pimpalwar, M.D., Interventional Radiologist at UT Southwestern, to see if the thoracic duct is open and if there are lymphatic collaterals causing effusions into the pleural spaces. The interventionalist team can then guide a Chiba needle through the intestines and essential organs, and then use glue to close the thoracic duct.

Our team is currently doing teleconsultations with physicians in several states to help evaluate whether lymphatic occlusion is appropriate for their Fontan patients.

“Our goal is to become the main lymphatic occlusion center serving patients across the Southern United States,” Dr. Reddy says.

He and his colleagues have completed eight of these procedures, with excellent results.

“In the old days, these patients would have moved to transplant and had all the bad outcomes that plastic bronchitis patients have after transplant,” Dr. Reddy says. “But this intervention helps them stay much healthier and keep their hearts for much longer.”

Improving the Standard of Care

One of our program’s key goals is to conduct research that will identify ways to improve the standard of care. Working in collaboration with other centers, we hope to: 

  • Determine the optimal age for Fontan surgery
  • Evaluate developmental outcomes following Fontan surgery
  • Evaluate non-cardiac related pathologies including Fontan-associated liver disease and Fontan-associated kidney disease
  • Develop exercise recommendations for Fontan patients
  • Evaluate the efficacy of pulmonary hypertension medications in Fontan patients

“There is still so much that we don’t know,” Dr. Reddy says. “Our goal is to answer some of these questions so we can not only develop a more comprehensive standard of care, but also find out how to help these patients live longer, healthier, more fulfilling lives.” 

Learn more about the innovative heart care and research at Children’s Health.

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