Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study.

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.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.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).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