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A Journal on Diseases of the Respiratory System

Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index




Minerva Pneumologica 2008 September;47(3):155-7

language: English

Severe pulmonary hypertension in adult congenital heart disease treated with Bosentan therapy

Actis Dato G. 1, Cappuccio G. 2, Zingarelli E. 1, Flocco R. 1, Forsennati P. G. 1, Hahn B. 2, Valesio R. 2, Casabona R. 1

1 Cardiac Surgery Department, Mauriziano Hospital, Turin, Italy
2 Cardiovascular Rehabilitation Clinic, Villa Serena Hospital, Piossasco, Turin, Italy


Bosentan is an antagonist of endothelin receptor used to treat pulmonary arterial hypertension. We present our initial experience in the treatment of Eisenmenger syndrome with Bosentan therapy. A significative New York Heart Associaton (NYHA) functional class reduction appeared with a relevant increase in walk distance and in oxygen saturation at rest. Transthoracic echocardiography (TTE) revealed an improvement of right ventricular performances with systolic pulmonary artery pressure (PAPs) and tricuspidal incompetence reduction. Tricuspid Anular Systolic Excursion (TAPSE), ejection time in pulmonary artery and acceleration time in pulmonary artery increased after 8 weeks. Eventually, with Bosentan we observed a good compliance and a reduction of other medications also of the oxygen delivery.

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