Abstract

Purpose: Monotherapy with bosentan (BOS), sitaxsentan (SIT), ambrisentan (AMB), sildenafil (SIL), or iloprost (ILO) is effective treatment for idiopathic PAH (IPAH) and connective tissue disease related (CT-PAH). However, few patients normalise functionally or hemodynamically on monotherapy, and combination therapy is then used. This study reports a National open label experience of varied, sequential, dual therapy.

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