Abstract
OBJECTIVES: Pulmonary arterial hypertension (PAH) is a rare disorder with unfavorable prognosis despite implementation of specific PAH-oriented therapy. The aim of the study was to define predictors of poor prognosis in patients from one center treated according to the Polish National Health Fund program. PATIENTS AND METHODS: Forty-seven consecutive patients (30 women; aged 39±17 years) with PAH diagnosis were enrolled to the study. Clinical assessment, laboratory measurements, electrocardiogram, echocardiography, 6-minute walk test, 24-hour Holter monitoring, cardiopulmonary exercise test and microvolt T-wave alternans test were performed during routine visits. Eight patients died during 2.6±1.7 years follow-up. RESULTS: Parametrs which differentiated patients who died were brain natriuretic peptide (BNP) concentration ≥330 pg/mL (sensitivity 88%, specificity 92%, area under the ROC curve [AUC] 0.92); bilirubin concentration ≥1.2 mg/dL (sensitivity 88%, specificity 81%, AUC 0.85); right atrial area ≥21 cm2 (sensitivity 86%, specificity 69%, AUC 0.84), right ventricular (RV) dimension in the apical 4-chamber view ≥47 mm (sensitivity 86%, specificity 86%, AUC 0.85) and RV to left ventricular diastolic diameter ratio ≥1.5 (sensitivity 83%, specificity 84%; AUC 0.85). In multivariate analysis, independent predictors of mortality were higher BNP (p = 0.04) and bilirubin level (p = 0.03), higher right atrial area (p = 0.02) and lower tricuspid annular plane systolic excursion (p = 0.03). CONCLUSIONS: In PAH patients treated with specific PAH-oriented therapy right atrial enlargement, impaired right ventricular systolic function, as well as increased BNP and bilirubin concentration was associated with an increased mortality risk.