The epidemiology of persons living with Fontan in 2020 and projections for 2030: Development of an epidemiology model providing multinational estimates

December 22, 2021
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Approximately 1 in 10,000 infants are born with a univentricular heart and are unlikely to survive into adulthood without the palliative Fontan procedure. In the modern era, the Fontan operation is the final operation of a staged surgical process (at least two major cardiovascular surgeries) conducted to achieve a Fontan circulation. Norwood is performed on infants in the first few weeks of life, followed by Glenn surgery at the age of around 4–6 months and, finally, the Fontan procedure is commonly performed in infants between the ages of 2 and 4 years. As a result of the surgery series, both superior and inferior venae cavae are rewired so that venous return is routed directly to the pulmonary artery, bypassing the heart, thus preventing oxygenated and deoxygenated blood from mixing in the single ventricle.

This study aimed to develop an epidemiologic model to estimate the prevalence of persons living with Fontan in 11 countries (Australia, New Zealand, Sweden, Switzerland, Netherlands, Germany, Italy, Spain, France, UK and USA). The prevalence was estimated for 2020 and extrapolated through to 2030, with stratification of prevalence estimates by procedure type (AP, LT-TCPC and EC-TCPC) and age group (\12 years, 12–17 years and C 18 years old).

This article was originally published in Advances in Therapy.

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