Study of Pulmonary Hypertension in Patients With Chronic Myeloproliferative Disorders at Sohag University Hospital

Study Overview
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Brief Summary
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Pulmonary hypertension (PH) is a complex condition that may be related to many clinical conditions. It is a serious disorder with a high morbidity and mortality rates. PH is classified into five groups according to clinical characteristics, pathological findings, hemodynamic characteristics and treatment response (Galie N, et al., 2016). These five groups include pulmonary arterial hypertension, PH due to left sided 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 (Simonneau G, et al., 2013). PH is a major complication of several hematologic disorders including myeloproliferative neoplasms (MPNs). 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), primary myelofibrosis (PMF) & chronic myeloid leukemia ( CML). Myeloproliferative neoplasms (MPNs) are included in group 5 PH (Arber DA, et al., 2016). This study will analyze the clinical and laboratory data of MPNs patients and correlate them with development of PH in these patients aiming to identify parameters that can predict PH in MPNs patients and thus, identifying MPNs patients at highest risk for PH 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

Official Title
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Study of Pulmonary Hypertension in Patients With Chronic Myeloproliferative Disorders at Sohag University Hospital

Conditions
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Chronic Myeloproliferative Disorders

Intervention / Treatment
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* Diagnostic Test: Echocardiography


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients over 18 years old. Patients diagnosed with polycythemia vera, essential thrombocythemia, primary myelofibrosis and chronic myeloid leukemia according to the 2016 WHO classification and attended the outpatient Hematology Clinic at Sohag University Hospital. Exclusion Criteria: Patients less than 18 years old. Patients with pre-existing pulmonary hypertension from any other cause. Patients with left sided heart disease e.g. left sided heart failure, left sided valvular diseases, cardiomyopathies, left ventricular systolic or diastolic dysfunction. Patients with chronic lung disease e.g. COPD & interstitial lung disease. Patients with chronic kidney disease. Patients with connective tissue diseases. Patients with congenital heart diseases.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| MPNs patients who have echocardiographic probability of PH.<br> | Diagnostic Test: Echocardiography<br>* Trans-thoracic Echocardiography<br>|
| MPNs patients who do not have echocardiographic probability of PH<br> | Diagnostic Test: Echocardiography<br>* Trans-thoracic Echocardiography<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Prevalence of pulmonary hypertension in chronic myeloproliferative disorders patients | Prevalence of pulmonary hypertension in chronic myeloproliferative disorders patients | 2 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Identification of independent different predictors of pulmonary hypertension in chronic myeloproliferative disorders patients | Identification of independent different predictors of pulmonary hypertension in chronic myeloproliferative disorders patients | 2 years |