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Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy*

Published online by Cambridge University Press:  16 December 2015

Brian S. Snarr ,
Stephen M. Paridon ,
Jack Rychik  and
David J. Goldberg
Brian S. Snarr
Affiliation:
The Cardiac Center, The Children’s Hospital of PhiladelphiaPhiladelphia, Pennsylvania, United States of America
Stephen M. Paridon
Affiliation:
The Cardiac Center, The Children’s Hospital of PhiladelphiaPhiladelphia, Pennsylvania, United States of America Division of Cardiology, Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Jack Rychik
Affiliation:
The Cardiac Center, The Children’s Hospital of PhiladelphiaPhiladelphia, Pennsylvania, United States of America Division of Cardiology, Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
David J. Goldberg*
Affiliation:
The Cardiac Center, The Children’s Hospital of PhiladelphiaPhiladelphia, Pennsylvania, United States of America Division of Cardiology, Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: Dr D. J. Goldberg, MD, Assistant Professor of Pediatrics, Division of Cardiology, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19010-4399, United States of America. Tel: +267-426-8143; Fax: +267-425-6108; E-mail: [email protected]
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Abstract

The Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.

Keywords

Congenital heart diseaseFontan procedurephysiologypulmonary vasodilator
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 8: HeartWeek 2015 , December 2015 , pp. 1489 - 1492
Copyright
© Cambridge University Press 2015 

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Footnotes

*

Presented at the Children’s Hospital of Philadelphia Cardiology 2015: 18th Annual Update on Pediatric and Congenital Cardiovascular Disease: “Challenges and Dilemmas”, Scottsdale, Arizona, United States of America, Wednesday February 11, 2015 – Sunday, February 15, 2015.

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