The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension

• Published in: The European respiratory journal Vol. 39; no. 3; pp. 589 - 596
• Main Authors: NICKEL, N, GOLPON, H, GREER, M, KNUDSEN, L, OLSSON, K, WESTERKAMP, V, WELTE, T, HOEPER, M. M
• Format: Journal Article
• Language: English
• Published: Leeds Maney 01-03-2012
• Subjects: Adult; Aged; Atrial Function, Right - physiology; Biological and medical sciences; Cohort Studies; Endothelin Receptor Antagonists; Epoprostenol - analogs & derivatives; Epoprostenol - therapeutic use; Exercise Test; Familial Primary Pulmonary Hypertension; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - mortality; Hypertension, Pulmonary - surgery; Lung Transplantation; Male; Medical sciences; Middle Aged; Natriuretic Peptide, Brain - blood; Oxygen - blood; Peptide Fragments - blood; Phosphodiesterase 5 Inhibitors - therapeutic use; Pneumology; Prognosis; Prostaglandins - therapeutic use; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Retrospective Studies; Severity of Illness Index; Treatment Outcome; World; Human; World Health Organization functional class; Organization; Prognosis; Respiratory disease; Health; Idiopathic; Cardiovascular disease; Survival; Artery; Pulmonary hypertension; Follow up study; Right heart; right heart catheterisation; Pneumology
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/09031936.00092311

Current guidelines for the treatment of patients with idiopathic pulmonary arterial hypertension (IPAH) recommend basing therapeutic decision-making on haemodynamic, functional and biochemical variables. Most of these parameters have been evaluated as risk predictors at the time of diagnosis. The aim of the present study was to assess the prognostic impact of changes in these parameters after initiation of targeted therapy. A cohort of 109 patients with IPAH who had undergone haemodynamic, functional and biochemical assessments at baseline and 3-12 months after initiation of pulmonary arterial hypertension (PAH)-targeted therapy, were followed for a median 38 months in order to determine predictors of mortality at baseline and during the course of their disease. Within the observation period, 53 (48.6%) patients died and four (3.7%) underwent lung transplantation. Kaplan-Meier estimates for transplantation-free survival were 92%, 67%, and 51% at 1, 3, and 5 yrs, respectively. Among baseline variables, 6-min walk distance, right atrial pressure, cardiac index, mixed-venous oxygen saturation (S(v,O(2))) and N-terminal-pro brain natriuretic peptide (NT-proBNP) were independent predictors of survival. During follow-up, changes in World Health Organization functional class, cardiac index, S(v,O(2)) and NT-proBNP proved significant predictors of outcome. When assigned to prognostic groups, improvements as well as deteriorations in these parameters after initiation of PAH-targeted therapy had a strong impact on survival. Measurements obtained at follow-up had a higher predictive value than variables obtained at baseline. Changes in established predictors of outcome during the course of the disease provide important prognostic information in patients with IPAH.