Incidence and outcome of pulmonary hypertension in myelofibrosis.

Abstract

e24001 Background: Myelofibrosis (MF) is a myeloproliferative neoplasm associated with an increased risk of cardiovascular disease, including pulmonary hypertension (PH). The prevalence, clinical characteristics, and outcomes of PH in MF remain poorly defined. This study aimed to evaluate the prevalence, risk factors, and clinical outcomes of PH in MF. Methods: A retrospective, single-center cohort study was conducted on 208 patients with MF diagnosed between 2013 and 2023. Inclusion criteria included (1) MF diagnosis per 5th edition of WHO classification, (2) somatic mutation profiling, and (3) echocardiographic data. PH was defined as right ventricular systolic pressure (RVSP) > 35 mmHg, estimated by tricuspid regurgitant jet. Major adverse cardiac events (MACE) were defined as new-onset HF, CAD requiring intervention, stroke/TIA, or cardiovascular death after MF diagnosis. Univariate and multivariable Cox proportional hazards models were used to evaluate overall survival (OS). Results: Echocardiograms were performed in 208 patients, with RVSP estimates available in 156 patients (75%). PH was present in 61 patients (39.1%). Patients with PH were older (65 vs. 62 years, p = 0.053) and had higher rates of baseline hypertension (65.6% vs. 43.2%, p = 0.01), atrial fibrillation (16.4% vs. 4.2%, p = 0.021), and cerebrovascular events (8.2% vs. 1.1%, p = 0.066). PH patients were less likely to undergo allogeneic stem cell transplant (23.0% vs. 58.9%, p < 0.001). MACE after diagnosis of MF occurred in 43 (20.7%) patients and was more frequent in PH patients (36.1% vs. 15.8% p = 0.007), predominantly driven by increased rates of new-onset HF (27.9% vs. 8.4%, p = 0.003). In univariate analysis, PH was associated with inferior OS (HR 1.83, 95% CI: 1.16-2.91, p = 0.009). However, in multivariable analysis adjusted for DIPSS Plus and allogeneic stem cell transplant status, PH was not independently associated with OS (HR 1.16, 95% CI: 0.70-1.92, p = 0.562). Conclusions: PH was prevalent among MF patients undergoing echocardiography and associated with increased cardiovascular morbidity, particularly HF. Although PH was not an independent predictor of mortality, its strong association with adverse cardiovascular events underscores the need for targeted screening and management in MF patients with cardiovascular risk factors.