AHA Statement Urges Holistic Approach to Fontan Patients

By Anne Harding

July 05, 2019

NEW YORK (Reuters Health) - Surveillance of children and adults with Fontan circulation should extend beyond the heart and blood vessels to other organ systems, and address the neurological and psychological challenges these patients face, according to a new scientific statement from the American Heart Association.

"Adult cardiologists are not all that familiar with this condition, because they've not had contact with these patients because they haven't existed before," Dr. Jack Rychik of The Children's Hospital of Philadelphia, who chaired the statement writing group, told Reuters Health by phone.

First introduced in 1968 by Francis Fontan to treat patients with tricuspid atresia, the operation is now used for "a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved," Dr. Rychik and colleagues explain in the statement, online July 1 in Circulation. More than 80% of patients who undergo the operation today can expect to survive for at least 30 years.

Because they lack a subpulmonary ventricle, Fontan-circulation patients experience high venous pressures and decreased cardiac output, which can lead to complications including circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy and plastic bronchitis, the authors note.

Patients may also have abnormalities in bone structure, body composition and growth, as well as liver fibrosis and renal dysfunction. "Cognitive, neuropsychological, and behavioral deficits are highly prevalent," the authors add.

Fontan-circulation patients may suffer from high levels of anxiety and traumatic stress, Dr. Rychik said. When they reach adulthood, poor learning skills and difficulties with executive function, along with physical health problems, may make it difficult for them to achieve independence, he explained. "They need to move on with their lives," he said. "Many don't have the capacity to do so."

Nevertheless, he added, "This is not all gloom and doom. We are understanding and characterizing what the challenges are with the goal being to increase the number of patients who are going to have a normal duration and quality of life."

The AHA statement includes proposed toolkits for surveillance testing of patients with Fontan circulation in childhood, adolescence and adulthood. While cardiovascular surveillance for these patients is well defined, Dr. Rychik noted, "we need to be looking at liver, kidney, neurocognitive outcomes in a serial manner in these patients."

He added: "Reacting to what are going to be complications is going to be much more expensive and costly to society than doing things to maintain the health of these patients."

Physical activity - once considered off-limits to patients with Fontan circulation - is an important way for them to improve and maintain their mental and physical health, Dr. Rychik said.

"What we're now learning is that exercise is probably the best medicine for these patients, and creating a culture of exercise early on . . . can pay benefits later in life and be very, very valuable," he added.

He and his colleagues conclude: "Although some studies show patients to have a decreased quality of life, others identify patients with Fontan circulation to have a quality of life similar to that of their peers. Even among some of the oldest patients, many attain higher levels of education, have jobs, and build their own families with satisfying and fulfilling lives."

"We are now entering a new phase in the management of patients born with single-ventricle CHD," they add. "Provided that patients undergo regular follow-up, adopt a healthy lifestyle, and are encouraged to participate in investigational clinical protocols and research, health-care providers and patients can share an optimistic view for a brighter future."

SOURCE: https://bit.ly/2XgNiDs

Circulation 2019.