@Article{chd.2024.052267,
AUTHOR = {Wenjie Dong, Zhibin Hong, Aqian Wang, Kaiyu Jiang, Hai Zhu, Fu zhang, Zhaoxia Guo, Hongling Su, Yunshan Cao},
TITLE = {Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {19},
YEAR = {2024},
NUMBER = {3},
PAGES = {325--339},
URL = {http://www.techscience.com/schd/v19n3/57465},
ISSN = {3071-1738},
ABSTRACT = { <b>Background:</b> Current guidelines for managing pulmonary arterial hypertension (PAH) recommend a risk stratification approach. However, the applicability and accuracy of these strategies for PAH associated with congenital heart disease (PAH-CHD) require further validation. This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up. Additionally, new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients. <b>Methods:</b> This retrospective study included 126 PAH-CHD patients. Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests. Independent risk factors were identified using ordered logistic regression, while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality. A new stratification model for the PAH-CHD population was constructed based on these analyses. <b>Results:</b> Significant survival differences across stratified risk groups were observed (<i>p</i> < 0.001), validating the effectiveness of the simplified risk stratification method in PAH-CHD patients. Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients (Hazard ratio 0.95, <i>p</i> < 0.001, C-index 0.70). A model combining N-terminal pro-brain natriuretic peptide, prothrombin activity, albumin, and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85. <b>Conclusions:</b> The simplified risk stratification method is applicable to PAH-CHD patients. Prothrombin activity is a strong independent predictor of adverse outcomes. A comprehensive risk stratification approach, incorporating both established and novel biomarkers, enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients, comparable to established models.},
DOI = {10.32604/chd.2024.052267}
}