BMPR2 and pulmonary arterial hypertension: Studies have suggested that patients with PAH carrying causal BMPR2 mutations present at an earlier age with more severe haemodynamic compromise.13, 14, 15, 16, 17 Although this might be expected to confer a worse survival, robust evidence describing the effect of BMPR2 mutations on long-term outcomes in these patients is lacking, primarily due to the limited power of individual studies and survival bias.18, 19