Prevalence and risk factors of high echocardiographic probability of pulmonary hypertension in myeloproliferative neopla

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ORIGINAL ARTICLE

Prevalence and risk factors of high echocardiographic probability of pulmonary hypertension in myeloproliferative neoplasms patients Yanika Jindamai1 · Ekarat Rattarittamrong1   · Arintaya Phrommintikul1 · Lalita Yongsmith1 · Pokpong Piriyakhuntorn1 · Thanawat Rattanathammethee1 · Sasinee Hantrakool1 · Chatree Chai‑Adisaksopha1 · Adisak Tantiworawit1 · Lalita Norasetthada1 Received: 29 April 2020 / Revised: 30 June 2020 / Accepted: 14 July 2020 © Japanese Society of Hematology 2020

Abstract Pulmonary hypertension (PH) is emerging as a complication of myeloproliferative neoplasms (MPNs). This was a prospective study conducted at Chiang Mai University Hospital. The primary objective was to determine the prevalence of high echocardiographic probability of PH in MPNs patients. The secondary objectives were to determine risk factors of PH and the correlation between risk factors and peak tricuspid regurgitation velocity ­(TRVmax). All MPNs patients aged over 18 years, including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), were enrolled. All eligible patients underwent echocardiography for evaluation of the probability of PH in line with the 2015 European Society of Cardiology (ESC) and the European Respiratory Society (ERS) Guidelines. Sixty-six patients with a median age of 59 years were enrolled. There were 35 PV, 25 ET, and 6 PMF patients included. The prevalence of high echocardiographic probability of PH was 4.55% (2 ET and 1 PMF). Risk factors associated with high echocardiographic probability of PHT were not analyzed. There were three risk factors associated with increased ­TRVmax, specifically lower hemoglobin, older age, and the presence of JAK2V617F mutation. The prevalence of high echocardiographic probability of PH in MPNs patients was 4.55%. Keywords  Pulmonary hypertension · Myeloproliferative neoplasms · Polycythemia vera · Essential thrombocythemia · Primary myelofibrosis

Introduction Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure (PAP) of at least 25 mmHg at rest by right heart catheterization [1]. There are 5 types of PH including pulmonary arterial hypertension, PH due to leftsided heart disease, PH due to lung disease and/or hypoxia, chronic thromboembolic pulmonary hypertension, or other pulmonary arterial obstruction and PH with unclear and/ or multifactorial mechanisms [1, 2]. Presenting symptoms Electronic supplementary material  The online version of this article (https​://doi.org/10.1007/s1218​5-020-02952​-4) contains supplementary material, which is available to authorized users. * Ekarat Rattarittamrong [email protected]; [email protected] 1



Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, 110 Inthawaroros Street, Muang, Chiang Mai 50200, Thailand

of PH are non-specific. Patients with PH may be asymptomatic in the early stages. Subsequently, patients can develop dyspnea on exertion, shortness of breath, fatigue, chest pain, syncope, or symptoms related to right-si