ABSTRACT

The early diagnosis require a high suspicious index, because the clinical picture is similar to other cardiovascular diseases.
The diagnostic approach includes two stages: detection (to determinate the nature of the clinical picture, establish the presence of pulmonary hypertension in high risk population) and characterization (to determine the specific clinical context, including causal factors, associated diseases, hemodynamic pattern and sequelae).
In symptomatic patients it is required a complete evaluation to identified a therapeutic strategy to reduce symptoms, improve the pulmonary vascular hemodynamics and increase survival. Otherwise, in non symptomatic patients is necessary to identify reversible causes and carefully monitoring of the course of the disease. This strategie permits to establish a non aggressive therapeutic approach (depending on severity of hemodynamics).
Once identified, by clinical evaluation, chest radiography, electrocardiogram and echocardiography. Right heart catheterization confirms the diagnosis, and documents initial hemodynamic pattern. Survival is determined by functional class, exercise capacity, hemodynamic evolution, response to reactivity test, right ventricular function, and biochemical parameters (brain natriuretic peptide, endotelin-1, troponins and uric acid). Long term evaluation and assessment must be done in specialized centers.

KEYWORDS

Idiopathic pulmonary arterial hypertension, pulmonary arterial hypertension, echocardiography, mean pulmonary arterial pressure, right heart catheterization.

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