Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (M.B., G.-P.D., M.A.G.).
Department of Cardiovascular Medicine, Division of Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Germany (M.B., G.-P.D.).
Circulation. 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544.
The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload. Management of the SRV remains an ongoing challenge because SRV dysfunction has implications on short- and long-term outcomes for all patients irrespective of underlying cardiac morphology. SRV dysfunction can be subclinical, underscoring the need for tertiary follow-up and timely management of target hemodynamic lesions. Catheter interventions and surgery have an established role in selected patients. Cardiac resynchronization therapy is increasingly used, whereas pharmacological therapy is largely empirical. Mechanical assist device and heart transplantation remain options in end-stage heart failure when other management strategies have been exhausted. The present report focuses on the SRV with its pathological subtypes, pathophysiology, clinical features, current management strategies, and long-term sequelae. Although our article touches on issues applicable to neonates and children, its main focus is on adults with SRV.
系统性右心室（SRV）在先天性心脏病中很常见，其解剖范围、适应性、临床表型以及多变但总体预后良好。最常见的 SRV 临床情况包括完全性大动脉转位伴以往心房转换修复、先天性矫正性大动脉转位、双入口右心室伴以往 Fontan 姑息治疗以及左心发育不良综合征伴 Norwood-Fontan 方案姑息治疗。与系统性左心室相比，SRV 预后良好的原因是多因素的，包括明显的纤维肌肉结构、形状和功能、冠状动脉供应不匹配、三尖瓣固有异常、固有或获得性传导异常以及不同的 SRV 对压力或容量超负荷的适应性。SRV 的管理仍然是一个持续的挑战，因为 SRV 功能障碍对所有患者的短期和长期结局都有影响，无论其基础心脏形态如何。SRV 功能障碍可能是亚临床的，这突出了需要进行三级随访和及时管理目标血液动力学病变。在选定的患者中，导管介入和手术具有既定的作用。心脏再同步治疗越来越多地被使用，而药物治疗在很大程度上是经验性的。在其他治疗策略用尽时，机械辅助装置和心脏移植仍然是终末期心力衰竭的选择。本报告重点介绍了 SRV 及其病理亚型、病理生理学、临床特征、当前管理策略和长期后果。虽然我们的文章涉及适用于新生儿和儿童的问题，但主要重点是患有 SRV 的成年人。
