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Pulmonary Arterial Hypertension in Hospitalized Patients With Polycythemia Vera (from the National Inpatient Database)

Published:December 18, 2020DOI:https://doi.org/10.1016/j.amjcard.2020.12.023

      Highlights

      • Patients with polycythemia vera have an increased prevalence of PAH.
      • Polycythemia vera is an independent predictor of PAH.
      • Patients with P. vera and PAH have an increased risk for in-hospital mortality.
      The prevalence and pathophysiology of pulmonary arterial hypertension (PAH) in myeloproliferative neoplasms are poorly understood and have not been evaluated on larger scale studies. These reports generally focus on all myeloproliferative neoplasms rather than each disease individually. We aim to establish the prevalence and association of PAH in patients with polycythemia vera (P. vera) using the Nationwide Inpatient Sample database. We identified 38,395 patients with a discharge diagnosis of P. vera and 39,395,521 patients without P. vera. Both groups were queried for a concomitant discharge diagnosis of PAH. Unadjusted and multivariate adjusted logistic regression analyses were performed to determine if presence of P. vera predicted PAH after adjusting for clinically relevant risk factors. The prevalence of P. vera was 0.1% of all hospital discharge records. Pulmonary arterial hypertension was more prevalent in patients with P. vera compared with the control population (7.9% vs 1.9%, p <0.0001). Presence of P. vera was a determinant of PAH in unadjusted analysis (odds ratio 4.4 [4.1 to 4.8]; p <0.001). After adjusting for age, gender, race, diabetes, tobacco use, previous history of tumor, previous history of pulmonary embolism, chronic lung disease, and chronic kidney disease, P. vera was associated with nearly a 3-fold higher risk for PAH after adjusting for several risk factors (2.98 [2.7 to 3.2], p <0.001).
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