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Prevalence of pulmonary hypertension in myelofibrosis

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Abstract

Pulmonary hypertension (PH) has been described in myelofibrosis (MF), but it is rare and typically found in advanced disease. Although the etiology of PH in MF is unclear, early predictors may be detected by echocardiogram. The goals of our study were to evaluate the prevalence of PH as determined by echocardiography in a cohort of MF patients and to identify clinical risk factors for PH. We performed a retrospective review of MF patients from October 2015 to May 2017 at MD Anderson Cancer Center in the ambulatory clinic, and those with echocardiogram were included. Clinical, echocardiographic, and laboratory data were reviewed. Patients with and without PH were compared using a chi-square or Fisher’s exact test, and logistic regression was performed with an outcome variable of PH. There were 143 patients with MF who underwent echocardiogram, and 20 (14%) had echocardiographic findings consistent with PH. Older age, male gender, hypertension, hyperlipidemia, coronary artery disease, dyspnea, hematocrit, brain natriuretic peptide (BNP), and N-terminal prohormone BNP (NT-proBNP) were significantly different between those without PH and those with PH (p < 0.05). Female gender was protective (OR 0.21, 95% CI 0.049–0.90, p = 0.035), and NT-proBNP was a significant clinical predictor of PH (OR 1.07, CI 1.02 = 1.12, p = 0.006). PH in MF is lower than previously reported in our MF cohort, but many patients had cardiac comorbidities. PH due to left-sided heart disease may be underestimated in MF. Evaluation of respiratory symptoms and elevated NT-proBNP should prompt a baseline echocardiogram. Early detection of PH with a multidisciplinary approach may allow treatment of reversible etiologies.

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Acknowledgments

We thank Dr. George A. Eapen for his assistance in the editorial review of this article.

Funding

This research is supported in part by the National Institutes of Health through MD Anderson Cancer Center Support Grant (CA016672).

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Authors and Affiliations

  1. Department of Cardiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Juan Lopez-Mattei, Cezar Iliescu, Saamir A. Hassan, Peter Kim & Nicolas L. Palaskas

  2. Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Srdan Verstovsek

  3. Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Bryan Fellman

  4. Division of Cardiovascular Medicine, McGovern Medical School at UTHealth, Houston, USA

    Karan Bhatti

  5. Divisions of Critical Care, Pulmonary and Sleep Medicine, McGovern Medical School at UTHealth, Houston, USA

    Brian A. Gray

  6. Department of Pulmonary Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Horiana B. Grosu & Saadia A. Faiz

  7. Keele Cardiovascular Research Group, Keele University, Keele, UK

    Mamas A. Mamas

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  1. Juan Lopez-Mattei
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Contributions

JL, SV, SF had full access to the all of the data in the study and takes responsibility for the integrity of the data and accuracy of the data analysis, including and especially any adverse effects. JL, SV, KB, BG, HG contributed substantially to the study design, data collection, data analysis and interpretation, and the writing of the manuscript. BF, CI, SH, PK, NP, and MM contributed to data analysis and interpretation, review and editing of the manuscript.

Corresponding author

Correspondence to Saadia A. Faiz.

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The study was conducted in accordance with the ethical rules of the Helsinki Declaration.

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Lopez-Mattei, J., Verstovsek, S., Fellman, B. et al. Prevalence of pulmonary hypertension in myelofibrosis. Ann Hematol 99, 781–789 (2020). https://doi.org/10.1007/s00277-020-03962-2

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