Introduction:

Pulmonary Arterial Hypertension (PAH) is a clinical condition that leads to failure in the right ventricle and death. The epidemiology of patients with pulmonary hypertension was characterized in a reference center in Uruguay.

Method:

52 patients with a diagnose of invasive pulmonary hypertension (January 2006-December 2016) were included in the study. Risk was estimated by means of a four variable model (functional class -FC, functional capacity, 6 minutes walking distance -6MWD, right atrium pressure and cardiac output).

Values of 1, 2 and 3 were allocated (low, medium and high risk respectively) to each variable (ESC/ERS 2015 PAH guidelines). Risk was categorized by rounding the average of the sum of values allocated for each variable to the nearest integer.

Results:

age ranged 46±2 years old, 85% of patients were women. Idiopathic hypertension, PAH associated to congenital heart disease (PAH-CHD) and PAH associated to connective tissue diseases (PAH-CTD) prevailed. Patients with PAH-CHD evidenced the greatest 6MWD and the lowest proportion of FC III/IV (p <0.05).

Survival was lowet in the PAH-CTD (p =0.069). Mortality after one year was 0%, 6% and 20% for patients with low (n=17), intermediate (n=28) and high (n=7) risk, respectively, regardless of age, sex and subgroup of PAH. 51% of patients with intermediate and high risk received combined treatment.

Conclusions:

the characteristics and survival of patients with PAH of a reference center in Uruguay were analysed in the study. The risk model allowed discriminating patient mortality. 51% of patients with intermediate and high risk received combined treatment.

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