State of the art of the Fontan strategy for treatment of univentricular heart disease

F1000Research
Jelle P G van der VenWillem A Helbing

Abstract

In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining chall...Continue Reading

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Citations

Nov 27, 2019·Cardiology in the Young·Aaron W EckhauserUNKNOWN Pediatric Heart Network Investigators
Nov 19, 2019·Acta Paediatrica·Marc GewilligJack Rychik
Mar 7, 2020·Journal of Cardiovascular Medicine·Gaetano D GargiuloGiuseppe Calcaterra
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Apr 12, 2019·Frontiers in Pediatrics·Fabian StöckerNicole Nagdyman
Mar 7, 2020·International Journal of Cardiology·Jelle P G van der VenWillem A Helbing
Jan 17, 2021·American Journal of Obstetrics & Gynecology MFM·Natasha K WolfeKathryn J Lindley
Feb 25, 2021·Journal of the American Heart Association·Eva van den BoschWillem A Helbing
Jul 25, 2020·Journal of the American College of Cardiology·Jiyong MoonMing-Sing Si
Apr 23, 2021·ASAIO Journal : a Peer-reviewed Journal of the American Society for Artificial Internal Organs·Joshua P CysykGerson Rosenberg

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