Pulmonary Hypertension Clinical Trial
Official title:
Screening for Pulmonary Vascular Changes in Patients With Chronic Myeloproliferative Diseases
Goal of the study is to assess the frequency of pulmonary hypertension in patients with
chronic myeloproliferative diseases. In each patient an echocardiography at rest will be
performed. In patients without musculoskeletal disease an exercise test (spiroergometry)
will be performed. Patients with elevated SPAP at rest or with reduced exercise capacity
(peak VO2 < 65%) a right heart catheterization (RHC) will be recommended. Also patients with
advanced NYHA functional class (III or IV) or with typical PH findings in electrocardiogram
will be advised to undergo a RHC. Additionally for the evaluation of exercise capacity a 6
MWD will be performed.
This work- up of patients allows clinical and hemodynamic evaluation.
Previous small studies and clinical cases have suggested a possible association between
pulmonary hypertension (PH) and chronic myeloproliferative disorders (CMPD). MPD may cause
PH through different mechanisms as: high cardiac output, asplenia, direct obstruction of
pulmonary arteries by megakaryocytes, chronic thromboembolic endothelial pulmonary
hypertension (CTEPH), porto-pulmonary hypertension (POPH). However, the exact prevalence of
PH in this group of disorders is not known.
This study is designed to identify the pulmonary vascular changes and describe the
prevalence of pulmonary hypertension (defined in this study as mean pulmonary arterial
hypertension (mPAP) ≥25mmHg as assessed by right-heart catheterization (RHC) or systolic
pulmonary arterial pressure (sPAP) ≥37mmHg (2.9 m/s) assessed by echocardiography.
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Observational Model: Cohort, Time Perspective: Prospective
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