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The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ventricular failure can usually be identified by a combination of clinical signs, symptoms, and imaging findings, diastolic ventricular dysfunction is likely an underrecognized cause of Fontan failure. Methods for detection of impaired diastolic function in a single ventricle are evolving, and established techniques appropriate in the biventricular heart lack validation in single ventricle. Association of biomarkers, cardiac magnetic resonance, and echocardiographic findings with invasively acquired pressure-volume loop data in humans may offer a way forward to accurate, noninvasive diagnosis. Today, therapy for severe diastolic ventricular dysfunction in the Fontan circulation is often disappointing and may require consideration of a ventricular assist device or even cardiac transplant. Progress toward improved outcomes of the Fontan palliation likely depends on successful innovation in primary prevention strategies and the development of more effective pharmacotherapy.
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Accepted for Publication: November 10, 2019.
Corresponding Author: Shelby Kutty, MD, PhD, MHCM, The Helen B. Taussig Heart Center, Johns Hopkins University School of Medicine, 1800 Orleans St, Baltimore, MD 21287 (email@example.com).
Published Online: February 5, 2020. doi:10.1001/jamacardio.2019.5459
Author Contributions: Drs Budts and Kutty had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Concept and design: Budts, Kutty.
Acquisition, analysis, or interpretation of data: Ravekes, Danford, Kutty.
Drafting of the manuscript: All authors.
Critical revision of the manuscript for important intellectual content: All authors.
Statistical analysis: Danford.
Administrative, technical, or material support: Budts, Ravekes, Kutty.
Supervision: Budts, Ravekes, Kutty.
Conflict of Interest Disclosures: Dr Budts reported receiving speech compensation from Abbott and Occlutech outside the submitted work. No other disclosures were reported.
Additional Contributions: Pieter De Meester, MD, PhD (Congenital and Structural Cardiology, University Hospitals Leuven, and Catholic University of Leuven, Leuven, Belgium), provided scientific input in the development of the artwork, and Fuad Zain, MBBS, MFA (HeartStudio, Rochester, New York), finished the graph works. There was no financial compensation outside of salary for these contributions.
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