Abstract Body (Do not enter title and authors here): Background: Myeloproliferative neoplasms (MPNs), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF), are chronic leukemias associated with cardiovascular (CV) disease (CVD), including pulmonary hypertension (PH). Prior studies utilizing echocardiography for identifying PH in MPNs have suggested an association between PH and adverse outcomes. However, right heart catheterization (RHC) is required for diagnosis and hemodynamic profiling of PH. Data on RHC-proven PH in MPNs are sparse.
Methods: We conducted a multicenter study of patients with MPN who underwent RHC. PH, defined as mean pulmonary artery pressure (mPAP) > 20 mmHg, was hemodynamically characterized into isolated pre-capillary (pre-cap), isolated post-capillary (post-cap), and combined pre- and post-capillary (Cpc-PH). Outcomes were heart failure hospitalization (HFH) or CV death, and all-cause death. Multivariable Cox proportional hazards regression was performed. To model total number of HFH, multivariable negative binomial regression was performed.
Results: 85 patients were included, 70 (82.4%) had PH, 37 (43.5%) were female, and 68 (80.0%) were White. Among PH patients, 30 (42.9%) had pre-cap PH, 12 (17.1%) post-cap PH, and 28 (40.0%) Cpc-PH. Age, gender, time from MPN to RHC, MPN type and driver and non-driver mutations did not differ between patients with or without PH. Patients with PH were more likely to have prior HFH (35.7% vs 0%, p = 0.006). After a median follow-up of 31.4 months, HFH or CV death occurred in 57 (67.1%) patients and death in 49 (57.6%). After adjustment, pre-cap (aHR 2.69, 95% CI 1.00 – 7.27) and post-cap PH were associated with increased risk of HFH or CV death (aHR 4.12, 95% CI 1.26 – 13.50) versus no PH. After multivariable negative binomial regression, any PH (IRR 3.36, 95% CI 1.31 – 8.63), pre-cap PH (IRR 4.11, 95% CI 1.60 – 10.58) and post-cap PH (IRR 5.06, 95% CI 1.63 – 15.67) were associated with increased total HFH.
Conclusions: Among patients with MPN who underwent RHC, PH is common and appears to be predominantly isolated pre-capillary and Cpc-PH. Isolated pre- and isolated post-capillary PH were associated with increased HFH. Our study is confounded by selection bias, and therefore prospective studies of PH as diagnosed with RHC are needed in MPNs.