Abstract：
BACKGROUND: Right ventricular (RV) imaging has not a definite role in risk stratification of pulmonary arterial hypertension (PAH) patients. We tested the hypothesis that echocardiography-derived phenotypes, depicting different degrees of RV remodeling and dysfunction, may provide additional prognostic information to current risk stratification tools.
METHODS: Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography. Simple echocardiographic variables were combined in order to define a priori four phenotypes representing different degrees of RV dilatation and RV-pulmonary arterial (PA) coupling: Phenotype 1 with mildy dilated right ventricle and preserved RV-PA coupling (n = 152 patients); phenotype 2 with mildly dilated right ventricle and poor RV-PA coupling (n = 143 patients); phenotype 3 with severely dilated right ventricle and preserved RV-PA coupling (n = 201 patients); phenotype 4 with severely dilated right ventricle and poor RV-PA coupling, with or without severe tricuspid regurgitation (n = 519 patients). Risk stratification was based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 3-strata model and Registry to Evaluate Early and Long-Term PAH disease Management (REVEAL) 2.0 score.
RESULTS: These phenotypes were present in all risk groups. Notably, regardless of the ESC/ERS risk stratum assigned to the patient, phenotype 4 was associated with a 2-fold increase of the odds of death (HR 2.1, 95% CI 1.6-2.8, p < 0.001), while phenotype 1 was associated with a 71% reduction in the odds of dying (HR 0.29, 95% CI 0.18-0.47, p < 0.001).
CONCLUSIONS: Echocardiography-derived phenotypes describing RV remodeling and dysfunction may provide prognostic information which is independent of and additional to the clinically defined risk in incident PAH patients.
METHODS: Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography. Simple echocardiographic variables were combined in order to define a priori four phenotypes representing different degrees of RV dilatation and RV-pulmonary arterial (PA) coupling: Phenotype 1 with mildy dilated right ventricle and preserved RV-PA coupling (n = 152 patients); phenotype 2 with mildly dilated right ventricle and poor RV-PA coupling (n = 143 patients); phenotype 3 with severely dilated right ventricle and preserved RV-PA coupling (n = 201 patients); phenotype 4 with severely dilated right ventricle and poor RV-PA coupling, with or without severe tricuspid regurgitation (n = 519 patients). Risk stratification was based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 3-strata model and Registry to Evaluate Early and Long-Term PAH disease Management (REVEAL) 2.0 score.
RESULTS: These phenotypes were present in all risk groups. Notably, regardless of the ESC/ERS risk stratum assigned to the patient, phenotype 4 was associated with a 2-fold increase of the odds of death (HR 2.1, 95% CI 1.6-2.8, p < 0.001), while phenotype 1 was associated with a 71% reduction in the odds of dying (HR 0.29, 95% CI 0.18-0.47, p < 0.001).
CONCLUSIONS: Echocardiography-derived phenotypes describing RV remodeling and dysfunction may provide prognostic information which is independent of and additional to the clinically defined risk in incident PAH patients.
摘要：
背景：右心室（RV）成像在肺动脉高压（PAH）患者的危险分层中没有明确的作用。我们检验了超声心动图衍生的表型，描绘不同程度的RV重塑和功能障碍，可能为当前的风险分层工具提供额外的预后信息。
方法：连续发生≥18岁的PAH患者，在2005年1月至2021年12月期间诊断，接受了临床评估，右心导管插入术,标准超声心动图。将简单的超声心动图变量组合以定义代表不同程度的RV扩张和RV-肺动脉（PA）偶联的四种表型：表型1，右心室轻度扩张，RV-PA偶联保留（n=152例）；表型2，右心室轻度扩张，RV-PA偶联不良（n=143例）；右心室重度扩张，右心室-PA偶联保留（n=201例）；有或没有严重的三尖瓣返流（n=519例）。风险分层基于ESC/ERS3层模型和REVEAL2.0评分。
结果：这些表型存在于所有风险组中。值得注意的是,无论分配给患者的ESC/ERS风险层如何，表型4与死亡几率增加2倍相关(HR2.1,95%C.I.1.6-2.8,p<0.001),而表型1与71％的死亡几率降低相关（HR0.29，95％C.I.0.18-0.47，p<0.001）。
结论：描述RV重塑和功能障碍的超声心动图衍生的表型可能提供独立于临床确定的PAH患者风险的预后信息。
方法：连续发生≥18岁的PAH患者，在2005年1月至2021年12月期间诊断，接受了临床评估，右心导管插入术,标准超声心动图。将简单的超声心动图变量组合以定义代表不同程度的RV扩张和RV-肺动脉（PA）偶联的四种表型：表型1，右心室轻度扩张，RV-PA偶联保留（n=152例）；表型2，右心室轻度扩张，RV-PA偶联不良（n=143例）；右心室重度扩张，右心室-PA偶联保留（n=201例）；有或没有严重的三尖瓣返流（n=519例）。风险分层基于ESC/ERS3层模型和REVEAL2.0评分。
结果：这些表型存在于所有风险组中。值得注意的是,无论分配给患者的ESC/ERS风险层如何，表型4与死亡几率增加2倍相关(HR2.1,95%C.I.1.6-2.8,p<0.001),而表型1与71％的死亡几率降低相关（HR0.29，95％C.I.0.18-0.47，p<0.001）。
结论：描述RV重塑和功能障碍的超声心动图衍生的表型可能提供独立于临床确定的PAH患者风险的预后信息。
