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

Abstract

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 (Sv,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, Sv,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.

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

“…These results were supported by comparable results for cardiovascular mortality in addition to correlations between RVEF and previously reported robust predictors of outcome in PAH, including cardiac index, right atrial pressure, TAPSE, NT-proBNP and SvO 2 [4,[20][21][22]. Collectively, these observations suggest measurement of RVEF may be a useful tool to evaluate the long-term prognosis of patients with PAH.…”

“…Changes in NYHA functional class, cardiac index, SvO 2 and NT-proBNP level better predict outcome than their baseline measurement [22,23]. Improvement in RVEF at 3-6 months after therapy was the best predictor of cardiovascular and overall survival.…”

“…Echocardiographic measurement of TAPSE at baseline predicted survival in a heterogeneous population of patients with precapillary PH (only one-third of whom had idiopathic PAH) [21]. Similarly, RVEF assessed with cardiac MRI independently predicted long-term outcome in patients with PAH [22].…”

The Role of Exercise Testing in the Modern Management of Pulmonary Arterial Hypertension

“…This was also demonstrable in a meta-analysis of the many treatment trials in PAH [141]. It retains significance in multivariate analyses even when other strong predictors are included such as patient demographics [127,140], baseline haemodynamics from right heart catheterisation or CMR [140,142,143], CPET variables [92,113] and presence of pericardial effusion [127]. Absolute values of 6MWD measured following PAH treatment retain prognostic power but do not improve on the baseline measurement [144].…”

Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‐1A.1)

“…On the contrary, long-term follow-up data show significant impact of therapy on PVR and CI rather than on PAP. The same applies when assessing the prognostic impact of changes in these parameters after initiation of targeted therapy [29]. Possibly, this reflects suboptimal dosing and/ or timing of escalation of therapy.…”