Risk factors associated with high probability of pulmonary hypertension in patients with myeloproliferative neoplasms

Myeloproliferative neoplasms (MPNs) are associated with pulmonary hypertension (PH). Recent studies have demonstrated associations between PH and adverse cardiovascular outcomes and MPN disease progression. However, risk factors for development of PH and the pathophysiology of PH in MPNs remain unclear. We conducted an analysis of a multicenter retrospective cohort of patients with MPN with ≥ 2 transthoracic echocardiograms (TTEs) after MPN diagnosis. Patients with low probability of PH on the first TTE after MPN diagnosis were included. Backward stepwise Cox proportional hazards regression modeling was performed to identify risk factors for high probability of PH on subsequent TTE. Impact of incident high probability of PH on subsequent TTE, on major adverse cardiovascular event (MACE), hematologic progression, and death was examined using multivariable competing risk or Cox proportional hazards regression. After backward stepwise modeling, age at MPN, non-JAK2 driver mutations, polycythemia vera (PV) and myelofibrosis (versus essential thrombocythemia), leukocytosis, and elevated HFA-PEFF score were associated with increased risk of incident high probability of PH. Incident high probability of PH was associated with increased risk of MACE, hematologic progression, and death. Further studies are needed to evaluate the role of prospective screening for PH in patients with MPNs and further delineate pathophysiology of PH in patients with MPNs.