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Published ahead of print on January 24, 2003, doi:10.1164/rccm.200210-1157BC

Am. J. Respir. Crit. Care Med., Volume 167, Number 8, April 2003, 1139-1141

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Submitted on October 12, 2002
Accepted on January 21, 2003

Sildenafil for long-term treatment of non-operable chronic thromboembolic pulmonary hypertension

Hossein A Ghofrani1, Ralph T Schermuly1, Frank Rose1, Ralph Wiedemann1, Markus G Kohstall1, Andre Kreckel1, Horst Olschewski1, Norbert Weissmann1, Beate Enke1, Stefanie Ghofrani1, Werner Seeger1, and Friedrich Grimminger1*

1 Internal Medicine, University Hospital, Justus-Liebig University Giessen, Giessen, Germany

* To whom correspondence should be addressed. E-mail: friedrich.grimminger{at}innere.med.uni-giessen.de.

Only a small percentage of patients with chronic thromboembolic pulmonary hypertension (CTEPH) are eligible for pulmonary thrombendarterectomy. We investigated the effects of oral sildenafil on hemodynamics and exercise capacity in 12 non-operable CTEPH patients. All patients were in disease progression despite sufficient long-term anticoagulation and best supportive care, and suffered from severe pulmonary hypertension (pulmonary vascular resistance index (PVRI) 1935+/-228 dyn*s*cm-5*m2, cardiac index (CI) 2.0 l*min-1*m-2, 6-min-walking distance 312+/-30 m). After approximately 6 months of sildenafil treatment, pulmonary hemodynamics and exercise capacity improved significantly (PVRI 1361+/-177 l*min-1*m2 (p=0.004), CI 2.4+/-0.2 l*min-1*m-2 (p=0.009), 6-minute-walking distance 366+/-28 m (p=0.02)). Therefore, oral sildenafil may offer a new option for medical treatment of this devastating disease.
Key words: Pulmonary hypertension, chronic thromboembolism, phosphodiesterase inhibitor, sildenafil




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