Prospective Longitudinal Study of Patients With Idiopathic Pulmonary Arterial Hypertension, Family or Taking Anorectics (EFORT)
The objective of this clinical research is to analyze the survival of a cohort of patients newly diagnosed (incident cases) with idiopathic PAH, familial or associated with the use of anorectics (isolated pulmonary vascular disease without comorbidity) and identify prognostic factors using a dynamic model for predicting survival, including prognostic factors evaluated repeatedly at pre-specified periods during follow-up. In a second step, the investigators define using this model combinations of parameters to better define the therapeutic goals in PAH (functional class, exercise testing, hemodynamic, echocardiographic variables, biological parameters).
|Study Design:||Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Prevention
|Official Title:||Evaluation of Prognostic Factors and Therapeutic Targets in Pulmonary Arterial Hypertension|
- death frequency [ Time Frame: 12 months ] [ Designated as safety issue: No ]
- death frequency [ Time Frame: Evolution between baseline assessment and follow-up. ] [ Designated as safety issue: No ]
A dynamic model for predicting survival will be used, including prognostic factors evaluated repeatedly at pre-specified periods during follow-up:
- Right Heart Catheterization: mean pulmonary arterial pressure at rest or during exercise, and pressure pulmonary artery occlusion
- Echocardiographic variables
- Biomarkers (brain natriuretic peptide(BNP), N Terminal Pro BNP, Big-endothelin, Isoprostanes)
- Functional Class of the New York Heart Association(NYHA)
- Walk Test 6 minutes
|Study Start Date:||January 2011|
|Estimated Study Completion Date:||January 2016|
|Estimated Primary Completion Date:||January 2016 (Final data collection date for primary outcome measure)|
Experimental: pulmonary hypertension
cohort of patients with pulmonary hypertension
Procedure: Right Heart Catheterization
all patients of the all centers will have Right Heart Catheterization at the diagnosis
Other Name: Right Heart Catheterization
Pulmonary arterial hypertension (PAH) is a rare disease characterized by an intense proliferation of pulmonary arterial wall causing increased progressive pulmonary vascular resistance, leading to right heart failure and death. Established prognostic factors at diagnosis were identified 20 years ago at a time when there is no specific treatment for describing the natural history of disease.
Over the last 10 years, new therapeutic classes (similar to prostacyclin, antagonists of endothelin receptors, inhibitors of phosphodiesterase 5) have been developed and improved symptoms, exercise capacity, and hemodynamics in patients with PAH. With the availability of these new molecules, the clinician is now faced with difficult treatment decisions regarding the choice of initial treatment and the need for road treatments combined during evolution. Therapeutic purpose and effect of these different therapeutic strategies on the long-term survival remain poorly understood.
If it has been clearly demonstrated that clinical parameters (NYHA functional class), functional (test 6-minute walk) and hemodynamic (cardiac output and pulmonary vascular resistance) measured before initiation of treatment have a major role in determining the prognosis, with the contribution of new molecules is important to evaluate the prognostic value of changes in these factors during follow-up under specific treatment. At the baseline assessment, including repeat cardiac catheterization rights, it is also important to evaluate other prognostic criteria substitution, including methods of noninvasive evaluation (echocardiography, biomarkers).
Please refer to this study by its ClinicalTrials.gov identifier: NCT01185730
|Le Kremlin Bicetre, France, 94275|
|Principal Investigator:||Olivier SITBON, MD, PhD||Assistance Publique - Hôpitaux de Paris|