Screening
Screening is indicated in patients with known genetic mutations predisposing to pulmonary hypertension, specifically the bone morphogenetic protein receptor type 2 (BMPR2) mutation, and in first-degree relatives of patients with heritable pulmonary arterial hypertension (PAH).[3] Doppler echo to detect elevated pulmonary artery pressure is the test of choice. Screening for PAH is also indicated in patients with the scleroderma spectrum of disease, and in patients with liver cirrhosis undergoing evaluation for liver transplantation. However, given the low prevalence of PAH, screening runs the risk of false-positives, and thus results must be carefully evaluated, ideally at expert centres.
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