Guidance Issued for Management of Fontan Circulation
More patients surviving to adulthood; management affected by scarcity of knowledge
TUESDAY, July 2, 2019 (HealthDay News) -- A scientific statement with recommendations for follow-up care in patients with Fontan circulation surviving into adulthood was published online July 1 in Circulation.
Jack Rychik, M.D., from the Children's Hospital of Philadelphia, and colleagues summarized the current state of knowledge on Fontan circulation and its consequences and provided recommendations for follow-up care.
The authors note that an increasing number of patients with Fontan circulation are surviving into adulthood, and managing their condition is impacted by a scarcity of medical knowledge. Potential complications involve the heart and multiple organ systems, such as the liver, lungs, brain, bones, and lymphatic system. Appropriate medical care for these patients includes a coordinated input from pediatric and adult congenital cardiologists and multiple subspecialists. As patients reach adulthood, many of the cardiac complications become more common. Management of other medical or surgical problems may be complicated by underlying Fontan physiology. Counseling on pregnancy may be challenging as few data exist on the long-term cardiovascular impact of pregnancy on Fontan physiology. Patients with Fontan circulation should be offered surveillance testing or systematic evaluation; for patients who are unwell, testing should be individualized. The optimal surveillance testing elements and frequency of testing should change as patients move toward and into adulthood.
"Health care providers, both pediatric and adult, will need to increase their understanding and knowledge of this unique cardiovascular condition in order to maintain and improve their quality of life," Rychik said in a statement.
Several authors disclosed financial ties to the pharmaceutical industry.