肺静脉封闭性疾病的结果和风险评估
Athénaïs Boucly1,2,3, Sabina Solinas1,2,3, Antoine Beurnier1,2,4
1Université Paris-Saclay, Faculté de Médecine, Le Kremlin-Bicêtre, France.
肺静脉封闭性疾病 (PVOD) 的生存率很低,治疗策略不会显著影响结果. 风险评估工具可以帮助预测PVOD患者的生存率.
科学领域:
- 心脏病学 心脏病学
- 肺部病理学 肺部病理学
- 罕见疾病 罕见疾病
背景情况:
- 肺静脉封闭性疾病 (PVOD) 是肺动脉高血压 (PAH) 的一种罕见而严重的形式.
- 目前欧洲心脏病学会/欧洲呼吸学会 (ESC/ERS) 的指导方针建议评估PAH的严重程度,但对于PVOD没有任何风险评估方法得到验证.
- 这项研究解决了对PVOD的验证风险评估的需求.
研究的目的:
- 确定PVOD患者的预后因素.
- 检查不同治疗策略对PVOD存活率的影响.
- 评估PVOD现有的风险评估方法的准确性.
主要方法:
- 来自法国肺高血压登记处的327名PVOD患者的分析 (2006-2021年).
- 基于初始治疗和风险状况的生存评估,与匹配的PAH患者相比.
- 应用和比较六种风险评估方法 (例如,ESC/ERS模型,REVEAL 2.0) 使用哈雷尔的c统计.
主要成果:
- 在1,3年和5年的PVOD生存率分别为86%,50%和27%.
- 只有6分钟的步行距离是显著的预后因素;治疗策略没有影响生存率.
- 所有经过测试的风险评估方法都预测了死亡率,ESC/ERS四层模型显示了最高的准确性 (基线C指数为0.64,随访时为0.74).
- 在可比风险层中,PVOD患者的生存率低于PAH患者.
结论:
- PVOD与预后不佳有关,初始治疗选择不会显著改变存活率.
- 风险评估工具对于预测PVOD患者的生存率是有价值的.
- 该ESC/ERS四层模型证明了PVOD风险分层的实用性.
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