Evidence of Pulmonary Hypertension in Myeloproliferative Neoplasm

Autor: Yaylali, YT, Yilmaz, S, Akgun-Cagliyan, G, Kilic, O, Kaya, E, Senol, H, Ozen, F
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Popis: Background:Pulmonary hypertension (PH) can complicate the course of myeloproliferative neoplasms (MPNs). Echocardiography is a useful noninvasive screening test for PH in populations at risk. We aimed to investigate the echocardiographic evidence of PH and clinical characteristics of patients with MPNs.Methods:This study included 197 patients with MPNs (mean age, 59 +/- 14 years; females, 53%; mean disease duration, 3.4 +/- 2.8 years). Clinical and laboratory characteristics, including JAK2V617F mutation status, were obtained. All participants underwent a comprehensive transthoracic echocardiographic examination. The echocardiographic evidence of PH was defined as systolic pulmonary artery pressure (SPAP) >= 40 mm Hg.Results:Overall, 11 patients (5.5%) with SPAP >= 40 mm Hg had echocardiographic evidence of PH. Patients with myelofibrosis had echocardiographic evidence of PH more often than patients with other MPNs (p< 0.001). Disease duration since the diagnosis of MPNs was 6.7 +/- 4.6 years in the PH group and 3.1 +/- 2.5 years in the non-PH group (p< 0.001). There was a weak positive correlation between SPAP values and time since diagnosis (r= 0.236,p =0.001). JAK2V617F mutation was not associated with PH. In multivariate logistic regression analysis, the presence of myelofibrosis (odds ratio [OR]: 22.177, 95% CI: 4.480-109.790,p< 0.001), long disease duration (OR: 1.217, 95% CI: 1.024-1.447,p= 0.026), and high uric acid levels (OR: 1.868, 95% CI: 1.049-3.328,p= 0.034) were found to be related with the echocardiographic evidence of PH. Survival was worse in the PH group (p= 0.0001).Conclusion:Our results suggest that patients with myelofibrosis are more likely to develop PH than other MPNs patients. Disease duration may predict the development of PH in MPN patients. C1 [Yaylali, Yalin Tolga; Yilmaz, Samet; Kilic, Oguz; Ozen, Furkan] Pamukkale Univ, Fac Med, Dept Cardiol, Denizli, Turkey. [Akgun-Cagliyan, Gulsum] Pamukkale Univ, Fac Med, Dept Hematol, Denizli, Turkey. [Kaya, Emrah] Ardahan State Hosp, Div Cardiol, Ardahan, Turkey. [Senol, Hande] Pamukkale Univ, Fac Med, Dept Biostat, Denizli, Turkey.
Databáze: OpenAIRE