Department of Pediatric and Adult Congenital Cardiology, University Hospital of Bordeaux, Bordeaux, France; Electrophysiology and HeartModeling Institute, IHU Liryc, Fondation Bordeaux Université, Bordeaux, France; Centre de recherche Cardio-Thoracique de Bordeaux, Inserm, Bordeaux, France.
Department of Congenital and Pediatric Cardiology, M3C-Enfants Malades Hospital, AP-HP, Paris, France; University Paris Cité, Paris, France.
Arch Cardiovasc Dis. 2022 Aug-Sep;115(8-9):476-486. doi: 10.1016/j.acvd.2022.05.004. Epub 2022 Jul 25.
Systemic right ventricle (SRV) is commonly encountered in patients with congenital heart disease. This nomenclature includes diseases with different anatomic features, adaptation and clinical phenotypes, and has a variable - but overall guarded - prognosis. Right ventricular fibromuscular architecture, shape, adaptation to overload conditions, rhythmic disorders and - most of all - tricuspid regurgitation (TR) contribute to the pathophysiology of SRV failure. The pivotal role of TR is complex as it is due to both the intrinsic abnormalities of the valve (specific to each phenotype) and the consequence of SRV dilation and failure. Medical therapy has not been equivocally proven to be effective for TR. Surgery (valve repair or replacement) has shown conflicting long-term results, mainly dependent on preoperative SRV function. Thus, other management options have been proposed to improve SRV function and valve competency, such as early anatomical correction, pulmonary banding, resynchronization therapy and valvular edge-to-edge percutaneous repair. The aim of this review is to discuss the mechanisms of TR and SRV failure, as well as the available therapeutic options.
系统性右心室（SRV）在先天性心脏病患者中很常见。该命名法包括具有不同解剖特征、适应性和临床表型的疾病，其预后具有可变但总体保守的特点。右心室纤维肌肉结构、形状、对超负荷条件的适应性、节律紊乱以及最重要的三尖瓣反流（TR）有助于 SRV 衰竭的病理生理学。TR 的关键作用很复杂，因为它既与瓣膜的固有异常（与每种表型相关）有关，也与 SRV 扩张和衰竭的后果有关。目前尚无明确证据表明药物治疗对 TR 有效。手术（瓣膜修复或置换）的长期结果存在矛盾，主要取决于术前 SRV 功能。因此，已经提出了其他管理方案来改善 SRV 功能和瓣膜功能，例如早期解剖矫正、肺动脉环缩术、再同步治疗和瓣缘对缘经皮修复。本文旨在讨论 TR 和 SRV 衰竭的机制以及可用的治疗选择。
