Pulmonary arterial hypertension (PAH) is a severe, incurable condition often diagnosed late, with a reduced life expectancy. The prognostic value of echocardiographic parameters during therapy has been minimally studied. This study aims to analyze their utility over five years and compare them with established clinical parameters.
Methods:
A retrospective analysis was conducted on patients from the specialized pulmonary hypertension clinic at the University Hospital of Cologne. Data from more than 110 patients were assessed over a five-year follow-up period. The cohort was stratified into deceased (Group 1) and surviving patients (Group 0). Clinical, laboratory and echocardiographic measurements were carried out at baseline and after 1, 3 and 5 years. Kaplan-Meier survival analysis was employed to assess survival differences based on sex and pre-existing conditions, with group comparisons performed using the log-rank test.
Results:
At baseline, 236 patients were evaluated, 61 patients died during the observation period. Considering the total cohort only the VCI-diameter showed a significant difference at baseline, which appeared clinically irrelevant. In the subgroup analysis, Group 0 had a significantly longer 6MWD at baseline, while no other parameter showed significant differences at this time. After one year of therapy, significant improvements in clinical and echocardiographic parameters were observed in both groups. In Group 0, there was a significant reduction in NYHA class and VCI-diameter, a highly significant reduction in sPAP, a higher TAPSE/sPAP ratio, and a highly significant reduction in TRV. After three years, the groups showed divergent trends. In Group 0, NTproBNP was significantly lower, and the NYHA class significantly improved. RA-area was significantly smaller, and RV-FAC was significantly greater. Differences in sPAP, TAPSE/sPAP ratio, and TRV between the groups became highly significant. After five years, the difference in NTproBNP remained significant, while NYHA class, VCI-diameter, RA-area, RV-FAC, sPAP, TAPSE/sPAP, and TRV differed highly significantly. TAPSE was significantly larger in Group 0 for the first time compared to Group 1. The Kaplan-Meier survival analysis indicated significantly worse survival in male patients and those with DM-Typ II.
Conclusions:
Echocardiographic parameters are significantly correlated with mortality in PAH patients. Initially, a positive therapy response was measurable in both groups, followed by a deterioration of varying degrees. After one year, highly significant differences between the groups were found for TRV, and very significant differences for sPAP and the TAPSE/sPAP ratio, suggesting that these parameters provide the earliest prognostic indicators. By year three, significant differences emerged in RA-area and RV-FAC. TAPSE, however, only differed significantly after five years, suggesting it may be a later or less sensitive prognostic indicator compared to the other parameters. The prognostic importance of established markers, particularly NYHA class as a longitudinal parameter and 6MWD as a valuable baseline marker, was confirmed. The Kaplan-Meier analysis identified male sex and DM-Typ II as relevant prognostic factors for mortality. These findings could contribute to the improved identification of high-risk patients who may benefit from intensified therapy.
