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.

中文翻译：

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特发性肺动脉高压患者的右心室表型分析。


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。我们测试了这样的假设：超声心动图衍生的表型描述了不同程度的右心室重塑和功能障碍，可能为当前的风险分层工具提供额外的预后信息。方法 2005 年 1 月至 2021 年 12 月期间诊断的年龄≥18 岁的连续发生 PAH 患者接受临床评估、右心导管检查、标准超声心动图检查。组合简单的超声心动图变量，以先验地定义代表不同程度的 RV 扩张和 RV-肺动脉 (PA) 耦合的四种表型：表型 1 具有轻度右心室扩张和保留 RV-PA 耦合（n = 152 名患者）；表型 2 伴有右心室轻度扩张和 RV-PA 耦合不良（n = 143 名患者）；表型 3 右心室严重扩张且保留 RV-PA 耦合（n = 201 名患者）；表型 4，右心室严重扩张，RV-PA 耦合不良，伴或不伴严重三尖瓣反流（n = 519 名患者）。风险分层基于欧洲心脏病学会/欧洲呼吸学会 (ESC/ERS) 3 层模型和评估早期和长期 PAH 疾病管理登记处 (REVEAL) 2.0 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 都与死亡几率增加 2 倍相关（HR 2.1，95% CI 1.6-2.8，p < 0.001），而表型 1与死亡几率降低 71% 相关（HR 0.29，95% CI 0.18-0.47，p < 0.001）。 结论 超声心动图衍生的描述 RV 重塑和功能障碍的表型可能提供独立于临床定义的 PAH 患者风险的预后信息。