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Understanding unique features of Fontan circulation.
Delineate typical modalities of failure in adult patients surviving with Fontan circulation.
Provide a rational framework to approach diagnostics and therapeutic measures to Fontan failure.
The first surgical rerouting of systemic venous inflow to the pulmonary circulation without a pumping ventricle was pioneered (in humans) by Dr Francis Fontan in 1968, as a palliative approach in three adult patients with tricuspid atresia.1 After extensive surgical iterations, nowadays, lateral tunnel baffling or extracardiac total cavopulmonary connection (TCPC) represent the modern staged surgical treatment of congenital heart diseases (CHDs) unsuitable for biventricular circulation either due to single ventricular anatomical cluster or adverse anatomical constraints (atrial isomerism, straddling atrioventricular valves and others) (figure 1).2
Long-term survival of patients with Fontan circulation (FC) outgrew the expectations and ~95% survival rate at 10 years after Fontan surgery is reported with the more modern surgical innovations.3 However, such delicate circulatory compromise comes at a cost along the way, and a constellation of cardiac and non-cardiac complications is reported in this growing patient population.2 Large registry data report 10-year freedom from death, heart transplantation (HTx), Fontan takedown or conversion, protein-losing enteropathy (PLE), plastic bronchitis (PB), or New York Heart Association functional …
ECD and CC contributed equally.
Contributors ECD and CC—reporting, conception and design. GEA—planning, conduct, reporting, conception and design, and interpretation of data.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.
Author note References which include a * are considered to be key references.
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