Prospective Longitudinal Study of Patients With Idiopathic Pulmonary Arterial Hypertension, Family or Taking Anorectics

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).