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From the author: I would like to thank V. Cottin and co-workers for their interest in our recently published study [1]. Their comments emphasise our conclusion that the treatment of the underlying disease should be the favoured strategy.Since both patients did not have a reduced cardiac index, when treatment with sildenafil and bosentan was started the lack of a significant improvement was not really surprising. The two patients reported by V. Cottin and co-workers showed different reactions. Patient one deteriorated dramatically in World Health Organization (WHO) functional class and 6-min walk distance (6MWD) “despite” (or due …

Pulmonary hypertension-specific therapy in patients with chronic respiratory insufficiency