The Epidemiology of Persons Living with Fontan in 2020 and Projections for 2030: Development of an Epidemiology Model Providing Multinational Estimates

INTRODUCTION: Fontan surgery is a palliative procedure performed in children with a functionally univentricular heart. Improvements in surgical technique over the past 30 years have increased life expectancy in this rare population. However, the epidemiology of persons living with Fontan is poorly understood. This study aimed to estimate the 2020 and 2030 prevalence of persons living with a Fontan circulation in 11 countries across the US, Europe, Australia and New Zealand, by procedure type: [atriopulmonary connection (AP), lateral tunnel total cavopulmonary connection (LT-TCPC) or extracardiac total cavopulmonary connection (EC-TCPC)]; and age group: [children (< 12 years), adolescents (12–17 years), and adults (≥ 18 years old)] by building an epidemiologic model. METHODS: The annual number of Fontan surgeries by country in 2010–2020 were extracted from hospital or claims databases, via procedure codes. The epidemiology of persons living with Fontan was modelled by applying these surgery frequencies to mid-year populations from 1972 to 2020 and overlaying an uptake curve. A literature search identified: 30-day mortality rates, long-term survival, and median age at surgery. Averages of these estimates were inputted into the model to project prevalence in 2030. RESULTS: The number of persons living with Fontan in 2020 across the 11 countries was estimated to be 47,881 [66 people per million (ppm)], rising to 59,777 (79 ppm) by 2030. In 2020, this population was 55% adults, 17% adolescents and 28% children shifting to 64%, 13% and 23%, respectively, in 2030. Among all persons living with Fontan, 74%/18%/9% are estimated to have EC-TCPC/LT-TCPC/AP, respectively, in 2020, and 83%/14%/4% in 2030. CONCLUSIONS: According to this epidemiology model, the Fontan population is growing, partly driven by increased survival rates with the more recent LT-TCPC and EC-TCPC procedures (compared with AP). The 2020/2030 prevalence of persons living with Fontan is 66/79 ppm. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12325-021-02002-3.