Early Recognition of Pulmonary Arterial Hypertension in Myelodysplastic and Myeloproliferative Diseases

Study Overview
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Brief Summary
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Myelodysplastic and myeloproliferative Disease represent conditions with increased risk for pulmonary hypertension. However, the exact prevalence of pulmonary hypertension in these conditions is not known. The effects of pulmonary hypertension on the clinical picture and the symptoms of patients in these conditions needs also further exploration. This exploratory study is designed to describe the prevalence of pulmonary hypertension in the population with such hematologic diseases, and the stages of pulmonary hypertension as well its effect on exercise capacity at time of diagnosis.

Detailed Description
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For early recognition of pulmonary hypertension exercise doppler echocardiography will be used in all patients. Patients with elevated pulmonary arterial pressure at rest or during exercise (estimated by echocardiography), or with decreased exercise capacity (as a potential sign of pulmonary hypertension) are advised to undergo right heart catheterisation. Cardiopulmonary exercise testing and six-minute walk distance measurement are performed to measure exercise capacity. The described work-up of patients allows precise and objective hemodynamic and clinical evaluation.

Official Title
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Early Recognition of Pulmonary Arterial Hypertension in Myelodysplastic and Myeloproliferative Diseases

Conditions
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Myeloproliferative Disorder, Myelodysplastic Syndromes, Pulmonary Hypertension

Intervention / Treatment
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* Other: echocardiography, right heart catheterisation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: myelodysplastic disease or myeloproliferative diseases Exclusion Criteria: known pulmonary hypertension relevant pulmonary disease relevant left cardiac or valvular disease recent major operations recent changes in medications relevant anaemia inability to exercise

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 95 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 |
| --- | --- |
| myeloproliferative, -dysplastic disease<br> | Other: echocardiography, right heart catheterisation<br>* at each patient an echocardiography will be performed at rest and during exercise. For the evaluation of exercise capacity, cardiopulmonary exercise testing and six-minute walk is performed. Right heart catheterisation is recommended to those with suspected pulmonary hypertension.<br>* Other names: EDE, RHC;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| mean pulmonary arterial pressure at rest and during exercise |  | at baseline and after 1 year | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| exercise capacity |  | at baseline and after 1 year |