Chest · 2026
Phenotyping of Pulmonary Arterial Hypertension Associated With Congenital Heart Disease Using Latent Class Analysis: Insights From a National Prospective Registry.
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Li Qimou, Yu Qingyun, Zhang Gangcheng, Tian Hongyan, Yu Zaixin, Zhang Caojin, Chen Yucheng, Liu Yuhao, Wu Weifeng, Zhu Xianyang, Zhu Xiulong, Yang Zhenwen, Xiong Changming, Han Huijun, Wu Yanqing, Gu Qing, He Jianguo
Аннотация
BACKGROUND: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is characterized by substantial prognostic and therapeutic heterogeneity. Conventional clinical and anatomical-pathophysiological classifications fail to fully capture these intricacies. Unsupervised clustering approaches such as latent class analysis (LCA) have been used to address this limitation.
RESEARCH QUESTION: Can LCA-derived phenogroups refine risk stratification and predict PAH-targeted treatment responses in adult patients with PAH-CHD?
STUDY DESIGN AND METHODS: Adult patients with PAH-CHD (N = 889) from a multicenter prospective registry were stratified into post- and pre-tricuspid shunt subsets. LCA was performed in each subset using 28 candidate clinical, laboratory, and hemodynamic variables. Ten-year survival and the effect of PAH-targeted therapy across phenogroups were evaluated.
RESULTS: LCA identified 4 phenogroups in each subset with distinct phenotyping patterns. In post-tricuspid shunts, Group 1 ("adaptation with open shunt") exhibited preserved right ventricular function and the best survival despite high afterload. Group 3 ("maladaptation and failure") and Group 4 ("closed shunt and failure") faced 2- to 3-fold higher mortality than Group 1. Notably, Group 2 ("hyperkinetic state") uniquely benefited from combination therapy (Pinteraction = .026). In pre-tricuspid shunts, Group 1 ("high shunt and mild resistance") and Group 3 ("young female and low inflammation") exhibited preserved survival despite varying afterloads. However, Group 2 ("old male and comorbidities") had significantly higher mortality and an attenuated treatment response (Pinteraction = .037) despite sharing mild hemodynamics with Group 1. Group 4 ("high resistance and failure") faced severe hypoxemia and the worst prognosis.
INTERPRETATION: LCA successfully stratified patients with PAH-CHD into distinct phenogroups with varied clinical profiles, survival, and treatment responses. This data-driven phenotyping system provides a novel approach for risk stratification and may guide personalized clinical decision-making.
CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT01417338; URL: www.
CLINICALTRIALS: gov.
RESEARCH QUESTION: Can LCA-derived phenogroups refine risk stratification and predict PAH-targeted treatment responses in adult patients with PAH-CHD?
STUDY DESIGN AND METHODS: Adult patients with PAH-CHD (N = 889) from a multicenter prospective registry were stratified into post- and pre-tricuspid shunt subsets. LCA was performed in each subset using 28 candidate clinical, laboratory, and hemodynamic variables. Ten-year survival and the effect of PAH-targeted therapy across phenogroups were evaluated.
RESULTS: LCA identified 4 phenogroups in each subset with distinct phenotyping patterns. In post-tricuspid shunts, Group 1 ("adaptation with open shunt") exhibited preserved right ventricular function and the best survival despite high afterload. Group 3 ("maladaptation and failure") and Group 4 ("closed shunt and failure") faced 2- to 3-fold higher mortality than Group 1. Notably, Group 2 ("hyperkinetic state") uniquely benefited from combination therapy (Pinteraction = .026). In pre-tricuspid shunts, Group 1 ("high shunt and mild resistance") and Group 3 ("young female and low inflammation") exhibited preserved survival despite varying afterloads. However, Group 2 ("old male and comorbidities") had significantly higher mortality and an attenuated treatment response (Pinteraction = .037) despite sharing mild hemodynamics with Group 1. Group 4 ("high resistance and failure") faced severe hypoxemia and the worst prognosis.
INTERPRETATION: LCA successfully stratified patients with PAH-CHD into distinct phenogroups with varied clinical profiles, survival, and treatment responses. This data-driven phenotyping system provides a novel approach for risk stratification and may guide personalized clinical decision-making.
CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT01417338; URL: www.
CLINICALTRIALS: gov.