@Article{chd.12526,
AUTHOR = {Gruschen R. Veldtman, Alexander R. Opotowsky, Samuel G. Wittekind, Jack Rychik, Daniel J. Penny, Mark Fogel, Bradley S. Marino, Marc Gewillig},
TITLE = {Cardiovascular adaptation to the Fontan circulation},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {12},
YEAR = {2017},
NUMBER = {6},
PAGES = {699--710},
URL = {http://www.techscience.com/schd/v12n6/39231},
ISSN = {3071-1738},
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.},
DOI = {10.1111/chd.12526}
}