High pulmonary hypertension risk by echocardiogram shortens survival in polycythemia vera

Pulmonary hypertension (PH) is a known complication of myeloproliferative neoplasms (MPNs) with an estimated prevalence as high as 50%. Patients with polycythemia vera (PV) report a wide spectrum of symptoms that significantly overlap with those reported by patients with PH. Yet, it is not known how PH affects outcomes and survival in patients with PV. To address this gap, we investigated the impact of echocardiography (ECHO)-based PH risk on survival of patients with PV from our large single-center cohort. Of 637 patients with PV, 134 had at least 1 ECHO and were included for analysis. Overall survival did not differ between patients who had or did not have ECHO. PH risk was established based on tricuspid regurgitation jet velocity. Kaplan-Meier analysis showed that high PH risk is associated with shortened survival compared with mild PH risk (median survival, 1.7 vs 3.7 years) or normal PH risk (median survival, not yet reached). Cox proportional hazard models found high PH risk was associated with a more than threefold increased risk of death, independent of age and thrombosis history. Logistic regression identified age (odds ratio, 6.9) and duration of PV diagnosis (odds ratio, 5.4) as significant risks for PH. Based upon these results and receiver operator characteristic optimization, we recommend echocardiographic screening for patients with PV aged >70 years or with duration of PV of >8 years. Further studies inclusive of invasive hemodynamics, advanced cardiovascular imaging, and MPN-associated biomarkers are needed to best characterize this group 5 PH population for therapeutic interventions.