Pulmonary Arterial Hypertension: Many Years' Experience and Modern Approach to a Malignant Disease in a Pulmonary Hypertension Centre

Introduction: The aim of this study was to record the results from a modern diagnostic and therapeutic approach to patients with pulmonary arterial hypertension. Methods: We studied the clinical characteristics and the treatment of 69 patients (50 women, 72.5%), aged 44 +/- 17 years, who were diagnosed with pulmonary hypertension (World Health Organisation categories 1, IV and V). The patients' outcomes were recorded over 14 years' operation of our Pulmonary Hypertension Unit. Results: Twenty-seven patients (39.1%) suffered from idiopathic pulmonary hypertension, 12 (17.4%) from thromboembolic obstructive disease, 14 (20.3%) from congenital heart diseases, 11 (15.9%) from connective tissue diseases, 3 (4.3%) from portal hypertension, 2 (2.9%) from sarcoidosis, and 1 (1.4%) from pulmonary veno-occlusive disease. Six patients (8.7%) were in New York Heart Association (NYHA) functional class I, 28 (40.6%) were in class II, 30 (43.5%) were in class III, and 5 (7.2%) were in class IV. Thirty-four patients completed a 6-minute walk test and covered a mean distance of 352 +/- 137 m. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were measured in 18 patients, with a mean value of 1665 1935 pg/ml. A vasoreactivity test in 41 patients had a positive response in 10 (24.4%). Twenty-six patients (37.7%) were treated with combination therapy, whereas 16 (23.2%) either did not comply with or were not given specific medication. The mortality over a mean follow-up period of 5 +/- 4 years was 26%, significantly lower than that reported before the introduction of new drugs. An advanced NYHA class was an independent predictor of mortality (p=0.004), while elevated NT-proBNP levels were also associated with poor survival (p=0.013). Conclusions: Pulmonary arterial hypertension is a severe disease that leads to right heart failure and death. Despite the latest advances, vigilance and continuous investigation are needed in order to achieve a prompt diagnosis and the most suitable treatment.