A recent review in Pediatric Cardiology provides insights into the Children’s Hospital of Philadelphia (CHOP) FORWARD program, a multidisciplinary model of care for patients with a Fontan circulation.
Single-ventricle congenital heart disease was once a lethal diagnosis for children, but over the past forty years, a series of procedures in the first years of life that reroutes blood flow through the heart has allowed children with this condition to survive. The resulting circulation, known as a Fontan circulation, allows blood flow to the body, provided by the single ventricle, while blood flow to the lungs occurs without the benefit of a pump. Blood flows to the lungs passively through staged surgery involving direct connections of the systemic veins to the vessels to the lungs, a cavopulmonary connection. Thousands of individuals with single ventricle around the world have survived this surgery and are now living with a Fontan circulation.
However, although the procedure allows for survival, individuals with this condition face many challenges. The altered blood flow leads to chronically elevated venous pressure and congestion, as well as relatively low cardiac output. As a result, patients often have numerous complications, ranging from cardiac issues like ventricular dysfunction and arrhythmia to other complications throughout the body, including the lymphatic system dysfunction, liver fibrosis, developmental delays, and behavioral and mental health problems.
To address the unique needs of this population, CHOP decided 10 years ago to launch the FORWARD program (Fontan rehabilitation, wellness and resilience development), a multidisciplinary clinic that focuses on the numerous issues patients with a Fontan circulation face.
“While our first hurdle of creating survival for most babies born with single ventricle heart disease is now achieved, our next goal is to fulfill a promise for a normal quality and duration of life for all with this condition” says Jack Rychik, MD, director of the CHOP FORWARD program. To that end, the FORWARD program was established, the first-of-its-kind clinic dedicated to comprehensive health for the individual with Fontan circulation.
Read more in this Pediatric Cardiology article, which describes the conception of the program and the experiences and perspectives that have shaped its development over the past 10 years.
Contributed by: Natalie Solimeo