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2 WU(IQR1.9-2.7). Baseline 6MWD was 352 m(IQR280-416) and 77% were in NYHA 3 or 4 functional class. All patients were commenced on initial monotherapy with an endothelin receptor antagonist(n=66) or phosphodiesterase-5 inhibitor(n=16). At first re-evaluation, 6MWD increased by 46 m(IQR7-96) and 35% demonstrated improvement in NYHA functional class. After a median follow-up of 65 months (IQR32-101), 18/82(22.0%) had died, with estimated 1-yr and 5-yr survivals of 98% and 84%, respectively. Death attributed to PAH occurr