Single ventricle heart defects are often fatal when left untreated and may require multiple surgeries. The Fontan procedure, performed around age 3, is the final stage of palliative surgical intervention for univentricular patients.
Arkansas Children’s Heart Institute is a center of excellence for single ventricle patients as well as a high-volume center for Fontan operations, according to Dala Zakaria, M.D., pediatric cardiologist, director of the single ventricle program at Arkansas Children’s Heart Institute and associate professor of pediatrics at the University of Arkansas for Medical Sciences.
Before the procedure was introduced five decades ago, most children with this condition did not survive into adulthood. But now, Dr. Zakaria said, "Our patients have an excellent survival rate following the procedure."
For patients at Arkansas Children's Heart Institute, Dr. Zakaria attributes the program's success to preoperative screening, optimizing hemodynamics and careful patient selection. An additional benefit for patients at Arkansas Children's Heart Institute is that the team follows a postoperative protocol that has resulted in an average hospital stay of nine days, significantly shorter than the benchmark of 14 days.
"We also have an excellent cardiac catheterization team that can intervene if needed following Fontan surgery," Dr. Zakaria said. For example, when treating a postoperative pleural effusion with a balloon pericardiotomy or fenestration, "our cardiac catheterization team performs the procedure, which helps decrease the length of hospital stay and gets those children home sooner with less morbidity."
Over the past three decades, long-term survival rates have improved significantly for children who undergo the Fontan procedure to improve circulation.
“Data show that their survival has improved from 69% before 1990 to close to 90% after 1990,” Dr. Zakaria said.
With the increased survival potential for Fontan patients, cardiologists at Arkansas Children's Heart Institute understood the need for life-long support. They developed the state's first Fontan program with a multidisciplinary team capable of monitoring for long-term complications. The program's goal goes beyond optimizing patients' physical health to supporting their mental health and quality of life. The Fontan program was structured to help children with Fontan circulation and their families by enabling patients to receive care from multiple specialists on the same day, minimizing their missed work and school days.
While necessary for many univentricular patients, Fontan circulation presents its own set of challenges. Fontan circulation is associated with long-term problems not limited to the cardiovascular system. Annual comprehensive screening of the liver, lungs, kidneys, endocrine and other systems are performed at the Fontan multidisciplinary clinic at Arkansas Children's Heart Institute, improving survival by assessing for any comorbidities that could affect patient outcomes.
Additional clinic objectives include optimizing patients’ circulation, supporting their neurodevelopmental progress and evaluating the need for behavioral therapy or medications — the last important because the last decade has brought greater recognition that patients with Fontan circulation may struggle with anxiety or academic difficulties.
"Since developing the Fontan program, we have found that many children experience neurodevelopmental problems or difficulty in school, so we are actively working to engage with their schools," Dr. Zakaria said. The Fontan clinic sends contact information and an educational package to schools attended by patients with Fontan circulation.
"We want these kids to be active, play sports and enjoy their lives," Dr. Zakaria said. "Our clinic goal is to improve both survival and quality of life. We provide them with all the resources that they need to live normal lives and be successful in school and work."
To enable a continuum of care from fetal management to adulthood, the clinic’s cardiology team includes specialists in treating adults with congenital heart issues.
The Fontan clinic at Arkansas Children's Heart Institute offers multisystem care for patients with Fontan circulation to improve early recognition and treatment of any long-term complications of the Fontan procedure. The team includes the following specialties:
Our pediatric heart team has the expertise to diagnose and treat all types of heart-related symptoms and conditions in kids.
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