The Long-Term Management of Children and Adults with a Fontan Circulation: A Systematic Review and Survey of Current Practice in Australia and New Zealand.

Although long-term survival is now the norm, Fontan patients face significant morbidity and premature mortality. Wide variation exists in long-term Fontan management. With an aim of improving their long-term management, we conducted a systematic review to identify best available evidence and gaps in knowledge for future research focus. We also surveyed cardiologists in Australia and New Zealand managing Fontan patients, to determine the alignment of current local practice with best available evidence. A systematic review was conducted using strict search criteria (PRISMA guidelines), pertaining to long-term Fontan management. All adult congenital and paediatric cardiologists registered with The Australia and New Zealand Fontan Registry were invited to respond to an online survey. Reasonable quality evidence exists for non-inferiority of aspirin over warfarin for thromboprophylaxis in standard-risk Fontan patients. No strong evidence is currently available for the routine use of ACE inhibitors, beta blockers or pulmonary vasodilators. Little evidence exists regarding optimal arrhythmia treatment, exercise restriction/prescription, routine fenestration closure, elective Fontan conversion and screening/management of liver abnormalities. Although pregnancy is generally well tolerated, there are high rates of miscarriage and premature delivery. Thirty-nine out of 78 (50 %) cardiologists responded to the survey. Heterogeneity in response was demonstrated with regard to long-term anti-coagulation, other medication use, fenestration closure and pregnancy and contraception counselling. Substantial gaps in our knowledge remain with regard to the long-term management of Fontan patients. This is reflected in the survey of cardiologists managing these patients. We have identified a number of key areas for future research.