PT  - JOURNAL ARTICLE
AU  - Roberta Pulcheri Ramos
AU  - Priscila Figueiredo
AU  - Eloara V.M. Ferreira
AU  - Carolina Messina
AU  - Rudolf Oliveira
AU  - J. Alberto Neder
AU  - Luiz Eduardo Nery
AU  - Jaquelina Sonoe Ota Arakaki
TI  - Riociguat improves excessive exercise ventilation in patients with inoperable chronic thromboembolic pulmonary hypertension
DP  - 2013 Sep 01
TA  - European Respiratory Journal
PG  - P2596
VI  - 42
IP  - Suppl 57
4099  - http://erj.ersjournals.com/content/42/Suppl_57/P2596.short
4100  - http://erj.ersjournals.com/content/42/Suppl_57/P2596.full
SO  - Eur Respir J2013 Sep 01; 42
AB  - Rationale: Excessive exercise ventilation in patients with CTEPH is thought to be mainly attributable to increased physiological dead space ventilation due to proximal obstructive thrombotic lesions. Accordingly, pulmonary endarterectomy has been shown to improve ventilatory responses during exercise. The impact of drug therapy on such alterations is still to be determined.Objective: To evaluate the effect of riociguat on the excessive exercise ventilation in patients with inoperable CTEPHMethods: 6 patients with inoperable CTEPH performed a symptom-limited ramp-incremental cardiopulmonary exercise testing (CPET) on a cycle ergometer before and 8-12 months after treatment with riociguat.Results: Long term treatment with riociguat significantly improved six minute walk distance (382 ± 63 to 431 ± 75m, p< 0.05). CPET demonstrated an improvement in ventilatory response to carbon dioxide output during exercise (ΔVE/ΔVCO2) from 65 ± 18 to 46 ± 7 (p< 0.05) and Peak PETCO2 (20 ± 4 to 26 ± 4mmHg, p <0.05), despite a non-significant difference in Peak O2 (861±169mL to 927± 141mL, p>0.05).Conclusions: Riociguat improves excessive exercise ventilation in patients with inoperable distal CTEPH.