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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| myeloproliferative, -dysplastic disease |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| echocardiography, right heart catheterisation | Other | 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. |
| Measure | Description | Time Frame |
|---|---|---|
| mean pulmonary arterial pressure at rest and during exercise | at baseline and after 1 year |
| Measure | Description | Time Frame |
|---|---|---|
| exercise capacity | at baseline and after 1 year |
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Inclusion Criteria:
Exclusion Criteria:
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patients with known myeloproliferative or myelodysplastic disease as a risk factor for pulmonary hypertension
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| Name | Affiliation | Role |
|---|---|---|
| Horst Olschewski, MD | Medical University of Graz, Pulmonology | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Medical University of Graz, Pulmonology | Graz | 8036 | Austria |
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| ID | Term |
|---|---|
| D009196 | Myeloproliferative Disorders |
| D009190 | Myelodysplastic Syndromes |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D001855 | Bone Marrow Diseases |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D008171 | Lung Diseases |
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Samples with DNA will be retained for later examinations at the Department for Genetics, if the patient agrees with it (specific question at the patient information).
|
|
| D012140 |
| Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |