BMPR2 mutations and survival in pulmonary arterial hypertension: An individual participant data meta-analysis

T Mnguni


Pulmonary arterial hypertension (PAH) is a rare disorder characterised by progressive remodelling of the small pulmonary arteries, resulting in increased pulmonary vascular resistance and ultimately right ventricular failure and death.[1,2] The diagnosis of PAH requires a mean pulmonary artery pressure of 25 mmHg or more with a pulmonary artery wedge pressure of 15 mmHg or less at right heart catheterisation in the absence of chronic thromboembolic, left heart or respiratory disease.

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