Abstract
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).
Abbreviations
- CVD:
-
Cardiovascular disease
- ET:
-
Essential thrombocythemia
- MF:
-
Myelofibrosis
- MPN:
-
Myeloproliferative neoplasms
- PAP:
-
Pulmonary artery pressure
- PH:
-
Pulmonary hypertension
- PV:
-
Polycythemia vera
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Funding
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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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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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). https://doi.org/10.1007/s12185-022-03454-1
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DOI: https://doi.org/10.1007/s12185-022-03454-1