Prevalence and Risk Factors of Pulmonary Hypertension in Patients with Myeloproliferative Neoplasms in Assiut University Hospital.

Overview

This study will evaluate the MPNs patients for the presence of PH by using a non invasive method ( Transthoracic echocardiography (TTE )) and correlate the clinical and laboratory data of these patients with development of PH aiming to identify risk factors for PH and parameters that can predict PH in MPNs patients and thus,identifying MPNs patients who require close monitoring \& screening for PH ,hoping that early detection and management of PH in MPNs patients can improve morbidity, prognosis and survival in those patients.

Pulmonary hypertension (PH) is a syndrome characterized by marked remodeling of the pulmonary vasculature and a progressive increase in the pulmonary vascular load. PH is a serious complex disorder that associated with high morbidity and mortality rates. PH is a complication of various hematologic diseases including myeloproliferative neoplasms (MPNs) which are included in group 5 ph. Myeloproliferative neoplasms (MPNs) are a group of diseases characterized by uncontrolled proliferation of at least one myeloid series due to an abnormal hematopoietic cell clone. There are different types of MPNs including polycythemia vera (PV), essential thrombocythemia (ET), chronic myeloid leukemia (CML), and primary myelofibrosis (PMF). PH is defined as an elevation in the mean pulmonary artery pressure (mPAP) that is greater than or equal to 25 mmHg, which was measured at rest via right heart catheterization (RHC). Systolic pulmonary artery pressure (sPAP) can be accurately estimated using Doppler transthoracic echocardiography (TTE), recent advances in noninvasive imaging led Doppler TTE to become the most widespread and well-recognized technique for non-invasive sPAP evaluation and the screening modality of choice for evaluating PH. Active monitoring of PH in patients with MPN may be warranted. Identification of risk factors predicting PH in MPN patients promises early detection and possibly improved PH management and therapy.

Conditions