Impact of Echocardiographic Probability of Pulmonary Hypertension on Prognosis and Outcomes Among Patients With Myeloproliferative Neoplasms

BACKGROUND: Myeloproliferative neoplasms (MPN) are a group of chronic leukemias that are associated with pulmonary hypertension (PH), which has been associated with increased risk adverse outcomes. The echocardiographic characterization of PH in MPN has not been reported, and the prognostic significance of PH among patients with MPN remains unclear. METHODS: Multicenter, retrospective cohort study of patients with MPN with ≥1 echocardiogram from 2010 to 2023. The echocardiographic probability of PH was determined according to the guidelines. The outcomes were hematologic progression and major adverse cardiovascular events. Exploratory analysis included outcomes among patients with right heart catheterization after the first echocardiogram, with PH defined as mean pulmonary artery pressure of >20 mm Hg. Multivariable Fine-Gray competing risk regression was used to estimate the subhazard ratio of hematologic progression and major adverse cardiovascular events. RESULTS: Five hundred fifty-five patients were included and 237 (42.7%) had an intermediate or high probability of PH on echocardiography. Over a median follow-up period of 51.2 months (interquartile range, 29.5–79.8), it was observed that echocardiographic probability of PH was associated with increased risk of hematologic progression (adjusted subhazard ratio, 1.92 [95% CI, 1.09–3.39]) and major adverse cardiovascular events (adjusted subhazard ratio, 1.66 [95% CI, 1.04–2.66]) but not all-cause death (adjusted hazard ratio, 1.51 [95% CI, 0.98–2.32]). Among patients with right heart catheterization (n=61), PH was present in 51 (83.6%) of patients and was associated with a higher risk of hematologic progression (29.4% versus 0%; P =0.048). CONCLUSIONS: Among patients with MPN, echocardiographic probability of PH was associated with an increased risk of hematologic progression. Prospective studies are needed to assess the optimal use of echocardiography on MPN-specific prognostication.