Open Access

REVIEW

Cardiovascular adaptation to the Fontan circulation

Gruschen R. Veldtman¹, Alexander R. Opotowsky², Samuel G. Wittekind¹, Jack Rychik³, Daniel J. Penny⁴, Mark Fogel³, Bradley S. Marino⁵, Marc Gewillig⁶

1 Adolescent and Adult Congenital Program, Heart Institute, Cincinnati Children’s Hospital Medical Centre, Cincinnati, Ohio, USA
2 Boston Children’s Hospital and Brigham and Women’s Hospital, Boston, Massachusetts, USA
3 The Cardiac Center at The Children’s Hospital of Philadelphia, Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA
4 Department of Cardiology, Texas Children’s Hospital and Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA
5 Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois, USA
6 Leuven University Hospital, Leuven, Belgium

* Corresponding Author: Samuel G. Wittekind, Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, MLC 2003, Cincinnati, Ohio. Email:

Congenital Heart Disease 2017, 12(6), 699-710. https://doi.org/10.1111/chd.12526

Abstract

Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide insight into the integrative pathophysiology that governs the Fontan circulation and stimulate thoughtful approaches to advance research.

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Keywords

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