Current risk stratification models for pulmonary arterial hypertension demonstrate c-statistics ranging from 0.56 to 0.83 in adults and 0.69 to 0.78 in children for predicting mortality.
Systematic Review (n=74)
Do risk stratification models accurately predict transplant-free survival or all-cause mortality in adult and pediatric patients with pulmonary arterial hypertension?
Current risk stratification models in pulmonary arterial hypertension demonstrate moderate to good prognostic discrimination for mortality, though robust validation in pediatric populations remains a critical gap.
Introduction: Currently, risk stratification is the cornerstone of determining treatment strategy for patients with pulmonary arterial hypertension (PAH). Since the 2015 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines for the diagnosis and treatment of pulmonary hypertension recommended risk assessment, the number of studies reporting risk stratification has considerably increased. This systematic review aims to report and compare the variables and prognostic value of the various risk stratification models for outcome prediction in adult and pediatric PAH. Methods: A systematic search with terms related to PAH, pediatric pulmonary hypertension, and risk stratification was performed through databases PubMed, EMBASE, and Web of Science up to June 8, 2022. Observational studies and clinical trials on risk stratification in adult and pediatric PAH were included, excluding case reports/series, guidelines, and reviews. Risk of bias was assessed using the Prediction model Risk Of Bias Assessment Tool. Data on the variables used in the models and the predictive strength of the models given by c-statistic were extracted from eligible studies. Results: = 9). The variables used most often in current risk stratification models were the non-invasive WHO functional class, 6-minute walk distance and BNP/NT-proBNP, and the invasive mean right atrial pressure, cardiac index and mixed venous oxygen saturation. C-statistics of current risk stratification models range from 0. 56 to 0. 83 in adults and from 0. 69 to 0. 78 in children (only two studies available). Risk stratification models focusing solely on echocardiographic parameters or biomarkers have also been reported. Conclusion: Studies reporting risk stratification in pediatric PAH are scarce. This systematic review provides an overview of current data on risk stratification models and its value for guiding treatment strategies in PAH. Systematic review registration: https: //www. crd. york. ac. uk/prospero/displayᵣecord. php? ID=CRD42022316885, identifier CRD42022316885.
Lokhorst et al. (Thu,) conducted a systematic review in Pulmonary arterial hypertension (n=74). Risk stratification models was evaluated on Prognostic value (c-statistic) for transplant-free survival or all-cause mortality. Current risk stratification models for pulmonary arterial hypertension demonstrate c-statistics ranging from 0.56 to 0.83 in adults and 0.69 to 0.78 in children for predicting mortality.