Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation | Zendy

Anders Hebert | Zendy; Ulla Ramer Mikkelsen | Zendy; Ulf Thilén | Zendy; Lars Idorn | Zendy; Annette Schophuus Jensen | Zendy; Edit Nagy | Zendy; Katarina Hanséus | Zendy; Keld E. Sørensen | Zendy; Lars Søndergaard | Zendy

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Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

Author(s) -

Anders Hebert,

Ulla Ramer Mikkelsen,

Ulf Thilén,

Lars Idorn,

Annette Schophuus Jensen,

Edit Nagy,

Katarina Hanséus,

Keld E. Sørensen,

Lars Søndergaard

Publication year - 2014

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.113.008441

Subject(s) - medicine , bosentan , cardiology , fontan procedure , physical therapy , heart disease , endothelin receptor , receptor

The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients.

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