The use of Macitentan in Fontan circulation: a case report

BMC Cardiovascular Disorders
Polyvios DemetriadesPaul F Clift

Abstract

The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation. We describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance. Macitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promi...Continue Reading

References

Feb 6, 2002·The Journal of Thoracic and Cardiovascular Surgery·Marilyne LévyFrancine Leca
Oct 30, 2004·The Journal of Thoracic and Cardiovascular Surgery·Max B MitchellJoseph R Coll
Jun 7, 2008·European Heart Journal·Alessandro GiardiniFernando Maria Picchio
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May 26, 2016·Heart·Marc Gewillig, Stephen C Brown

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Citations

Feb 29, 2020·American Journal of Physiology. Heart and Circulatory Physiology·Anastacia M GarciaStephanie J Nakano

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Methods Mentioned

BETA
pulmonary artery

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