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Pulmonary hypertension is associated with poor cardiovascular and hematologic outcomes in patients with myeloproliferative neoplasms and cardiovascular disease

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Cardiovascular events and hematologic progression to myelofibrosis or leukemia are leading causes of morbidity and mortality among patients with myeloproliferative neoplasms (MPN). Pulmonary hypertension (PH) is also associated with MPN and cardiovascular disease (CVD), though its prognostic significance in MPN is not well characterized. Our primary objective was to investigate the effect of PH, defined as right-ventricular systolic pressure (RVSP) ≥ 50 mmHg on echocardiogram or mean pulmonary artery pressure (mPAP) ≥ 20 on right heart catheterization, on cardiovascular and all-cause mortality and hematologic progression in patients with MPN and CVD (atrial fibrillation, heart failure hospitalization, and myocardial infarction after MPN diagnosis). Of the 197 patients included (86 ET, 80 PV, 31 PMF), 92 (47%) had PH and 98 (50%) were male. All-cause mortality (58 vs 37%, p = 0.004), cardiovascular death (35 vs 9%, p < 0.0001), and hematologic progression (23 vs 11%, p = 0.037) occurred more frequently in patients with PH. Multivariable competing-risk and proportional hazards regression showed that PH was associated with increased risk of all-cause death (adjusted hazard ratio [HR], 1.80, 95% CI 1.10–2.93), CV death (adjusted subdistribution HR 3.71, 95% CI 1.58–8.73), and hematologic progression (adjusted subdistribution HR 1.99, 95% CI 1.21–3.27).

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Data availability

Data are available on request to corresponding author (GH).



Cardiovascular disease


Essential thrombocythemia




Myeloproliferative neoplasms


Pulmonary artery pressure


Pulmonary hypertension


Polycythemia vera


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Dr. Orly Leiva received the American Society of Hematology Minority Resident Hematology Award Program grant for this research. The funding body had no role in the design of the study, data collection, analysis and interpretation of the data, and writing of the manuscript.

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Authors and Affiliations



OL, GH, and AB: conceived and planned the study. OL and AJ: performed chart review and data gathering. OL, SR, and DN: performed statistical analysis. OL, AB, RR, RKL, KG, and GH: wrote the manuscript. All authors read and approved the final manuscript.

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Correspondence to Gabriela Hobbs.

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The study was approved by the Massachusetts General Hospital Institutional Review Board. Research was carried out in accordance with the Declaration of Helsinki. The requirement of informed consent was waived by the Institutional Review Board.

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It is not applicable given no personal identifiable patient information included in publication.

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Leiva, O., Ren, S., Neuberg, D. et al. Pulmonary hypertension is associated with poor cardiovascular and hematologic outcomes in patients with myeloproliferative neoplasms and cardiovascular disease. Int J Hematol 117, 90–99 (2023).

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