Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms

Abstract

Pulmonary hypertension (PH) is a frequent complication of Philadelphia-negative myeloproliferative neoplasms (MPN), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). However, its prognostic significance is understudied. We, therefore, aimed to evaluate the effect of PH identified by echocardiography on the risk of progression to secondary MF or acute leukemia in MPN patients. We conducted a multicenter, retrospective cohort study of MPN patients with ≥1 echocardiogram from 2010-2023. PH was defined as pulmonary artery systolic pressure ≥40 mmHg. Outcomes were progression to secondary MF or leukemia, major adverse cardiovascular events (MACE) and allcause mortality. Multivariable Fine-Gray competing-risk regression analysis was used to estimate subdistribution hazard ratios (SHR) of hematologic progression and MACE. Five hundred and fifty-five patients were included (42.7% with PV, 41.1% with ET, 16.2% with MF) or whom 195 (35.1%) had PH. Over a median follow-up period of 51.2 months, PH was associated with an increased risk of secondary MF progression (adjusted SHR [aSHR], 95% confidence interval [95% CI]: 1.25-4.59), leukemia progression (aSHR=3.06, 95% CI: 1.13-8.25), and MACE (aSHR=1.59, 95% CI: 1.01-2.49) but not all-cause death (adjusted hazard ratio=1.48, 95% CI: 0.96-2.26). In patients with PH, absence of left heart disease was associated with a higher risk of secondary MF progression among patients with ET or PV (aSHR=2.76, 95% CI: 1.19-6.38) and leukemia progression among patients with MF (aSHR=7.18, 95% CI: 1.59-32.46). Prospective studies are needed to assess the role of echocardiography in MPN-specific prognostication.