With improving survival after the Fontan procedure, adult survivors need periodic cardiovascular and end-organ surveillance testing, according to a scientific statement from the American Heart Association (AHA).
Globally, the population of adults with Fontan circulation may have reached 20,000 to 28,000 in 2018, and that number is expected to double in the next 2 decades, noted writing group chair Jack Rychik, MD, of the Children's Hospital of Philadelphia, and colleagues in Circulation.
The writing group proposed a surveillance testing "toolkit" for those with Fontan circulation highlighting specific tests for affected organ systems at different ages. Surveillance testing should be offered every 1 to 2 years for those over the age of 18, every 1 to 3 years for those ages 12 to 18 years, and every 3 to 4 years for those under the age of 12 years.
Fontan circulation's characteristically decreased cardiac output and chronically increased systemic venous pressures lead to common complications of arrhythmia, plastic bronchitis, atrioventricular valve regurgitation, circulatory failure, ventricular dysfunction, and protein-losing enteropathy. Abnormalities in bone structure, growth, and body composition have been identified as well.
While there wasn't sufficient data for formal recommendations on specific tests or their frequency, "we believe that surveillance testing for cardiovascular and end-organ system consequences of the Fontan circulation is reasonable, clinically important, and to be encouraged as part of overall high-quality patient care," Rychik's group wrote.
Expert consensus recommendations in the statement included:
- It's reasonable to offer periodic systematic screening focused on cardiovascular and end-organ health to those who appear clinically well
- Individualize testing, especially for those that are unwell
- Optimal surveillance testing frequency and components change as the patient ages, as the rate of progression of dysfunctional changes, and as organ system complications arise
- Surveillance should be provided by healthcare centers and providers that have experience with Fontan circulation care and who are able to interpret results
Exercise may also be psychologically and physically beneficial for patients with Fontan circulation, the group noted.
The evaluation of the efficacy of pharmacological treatment is challenging due to the rarity of patients with Fontan circulation and heterogeneity. When it comes to clinical practice, there is variation in pharmacological treatment, they added.
The authors called for "interventional and preventive strategies" for this cohort, stating that although not fully tested, interventions aimed at neurodevelopmental outcomes, improved nutrition, and cardiac rehabilitation programs are promising. "Programs targeting patient education and health self-awareness may be of great value. Trials testing various medications are underway with anticipation of identifying optimal drug therapies to enhance and sustain cardiovascular and end-organ wellness," they emphasized.
"We are now entering a new phase in the management of patients born with single-ventricle CHD. Provided that patients undergo regular follow-up, adopt a healthy lifestyle, and are encouraged to participate in investigational clinical protocols and research, healthcare providers and patients can share an optimistic view for a brighter future," they concluded.
The role of treatments like heart transplantation, mechanical circulatory support, and cardiovascular drugs to treat and prevent complications remain unclear, the authors wrote.
"We need more research into the basic biology of single ventricle hearts and whether the damage to other organ systems, such as kidneys, liver and brain can be mitigated or reversed," said Rychik in a statement.
Rychik reported no disclosures.
Source Reference: Rychik J, et al "Evaluation and management of the child and adult with fontan circulation: A scientific statement from the american heart association" Circulation 2019.
Read the original article on Medpage Today: Post-Fontan Health Screening 'Toolkit' Proposed by AHA