Association of Disease Subtype and Duration with Echocardiographic Evidence of Pulmonary Hypertension in Myeloproliferative Neoplasm.

ABSTRACT

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.