Real-world prevalence and outcome of elevated right ventricular systolic pressure in myelofibrosis

BACKGROUND: Myelofibrosis (MF) is a subgroup of Philadelphia chromosome-negative myeloproliferative neoplasms that are associated with an increased risk of cardiovascular disease, including pulmonary hypertension. Here we report the real-world prevalence, risk factors, and clinical outcomes of eRVSP in MF patients. METHODS: A retrospective, single-center cohort study was conducted on 208 patients with MF that were diagnosed between 2013 and 2023. Elevated right ventricular systolic pressure (eRVSP) was defined as RVSP > 35 mmHg. Major adverse cardiac events (MACE) were defined as new-onset congestive heart failure, coronary artery disease requiring intervention, cerebrovascular events, or cardiovascular death after MF diagnosis. Univariate and multivariable Cox proportional hazard ratio regression model with eRVSP status as time-dependent covariate was used to estimate overall survival. RESULTS: Echocardiograms were performed in 208 MF patients, with RVSP estimates available in 156 patients (75%). eRVSP was present in 61 patients (39.1%). Patients with eRVSP 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 = 0.02). MACE after diagnosis of MF occurred in 43 (20.7%) patients and was more frequent in eRVSP patients (36.1% vs. 15.8% p = 0.007), and this was predominantly driven by increased rates of new-onset congestive heart failure (27.9% vs. 8.4%, p = 0.003). In multivariable analysis, the presence of time-dependent eRVSP was associated with reduced survival (aHR: 4.41; 95%CI:2.84-6.85) after adjustment for clinically relevant covariates. CONCLUSIONS: eRVSP was prevalent among MF patients undergoing echocardiogram and this was associated with increased cardiovascular morbidity, particularly HF. Furthermore, eRVSP was associated with inferior survival and underscores the need for targeted screening and management in MF patients with cardiovascular risk factors and diseases. Prospective studies with baseline cardiovascular assessment and echocardiograms in all MF patients will establish true prevalence and may further elucidate the morbidity and mortality implication of eRVSP in MF patients.