Abstract
Background: Right ventricular dysfunction is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to identify echocardiographic right heart parameters associated with adverse outcome and to develop a non-invasive, echocardiography-based risk score for PAH patients. Methods and Results: In 254 PAH patients we analyzed functional status, laboratory results, and echocardiographic parameters. We included these parameters to estimate all-cause death or lung transplantation using Cox regression models. The analyses included a conventional model using guideline-recommended variables and an extended echocardiographic model. Based on the final model a 12-point risk score was derived, indicating the association with the primary outcome within five years. During a median follow-up time of 4.2 years 74 patients died or underwent lung transplantation. The conventional model resulted in a C-Index of 0.539, whereas the extended echocardiographic model improved the discrimination (C-index 0.639, p-value 0.017). Ultimately, the newly developed risk score included WHO functional class, 6-min walking distance, N-terminal brain natriuretic peptide concentrations, pericardial effusion, right atrial area, tricuspid annular plane systolic excursion, and fractional area change. Conclusion: Integrating right heart function assessed by echocardiography improves prediction of death or lung transplantation in PAH patients. Independent validation of this finding is warranted.
Highlights
Most patients were in WHO functional class III or IV and had a severely reduced 6-min walk distance (6MWD) (269.2 m, standard deviation (SD) ± 183.9)
In this heterogenous but contemporary pulmonary arterial hypertension (PAH) cohort, we were able to confirm the predictive value of recognized clinical variables such as 6MWD, WHO functional class, and NT-proBNP levels [1], and identify less established variables like renal impairment and DLCO as predictors for adverse outcome in PAH
Similar to Sachdev and colleagues, we found a median Right ventricular (RV) free wall strain of −14.0%
Summary
The 2015 ESC/ERS guidelines for pulmonary hypertension (PH) introduced a risk evaluation tool to predict mortality at one year, allowing a classification of individuals in low (10%) categories [1]. Risk estimation in this model is based on clinical deterioration, functional lung capacity, brain natriuretic peptide (BNP), or. Methods and Results: In 254 PAH patients we analyzed functional status, laboratory results, and echocardiographic parameters We included these parameters to estimate all-cause death or lung transplantation using Cox regression models. Conclusion: Integrating right heart function assessed by echocardiography improves prediction of death or lung transplantation in PAH patients
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
