Lewis Matthew, Rosenbaum Marlon
Herbert Irving Pavilion, 161 Fort Washington Ave Suite 627, New York, NY, 10032, USA.
Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, NY, USA.
Curr Cardiol Rep. 2017 Aug;19(8):74. doi: 10.1007/s11886-017-0877-3.
Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the mitral valve, left ventricle, and aorta and is ultimately palliated with a single-ventricle repair. Universally fatal in infancy prior to the advent of modern surgical techniques, the majority of HLHS patients will now reach adulthood. However, despite improvements in early survival, the HLHS population continues to face significant morbidity and early mortality. This review delineates common sources of patient morbidity and highlights areas in need of additional research for this growing segment of the adult congenital heart disease population.
It has become increasingly clear that palliated adult single ventricle patients, like those with HLHS, face significant life-long morbidity from elevated systemic venous pressures as a consequence of the Fontan procedure. Downstream organ dysfunction secondary to elevated Fontan pressures has the potential to significantly impact long-term management decisions, including strategies of organ allocation. Because of the presence of a morphologic systemic right ventricle, HLHS patients may be at even higher risk than other adult patients with a Fontan. Because the adult HLHS population continues to grow, recognition of common sources of patient morbidity and mortality is becoming increasingly important. A coordinated effort between patients and providers is necessary to address the many remaining areas of clinical uncertainty to help ensure continued improvement in patient prognosis and quality of life.
左心发育不全综合征（HLHS）的特征是二尖瓣、左心室和主动脉发育不全，最终通过单心室修复进行姑息治疗。在现代外科技术出现之前，HLHS在婴儿期普遍致命，现在大多数HLHS患者能够活到成年。然而，尽管早期生存率有所提高，但HLHS患者群体仍然面临着显著的发病率和早期死亡率。本综述阐述了患者发病的常见原因，并强调了这一不断增长的成人先天性心脏病患者群体中需要进一步研究的领域。
越来越清楚的是，接受姑息治疗的成人单心室患者，如HLHS患者，由于Fontan手术导致体循环静脉压升高，面临着严重的终身发病风险。Fontan压力升高继发的下游器官功能障碍有可能显著影响长期管理决策，包括器官分配策略。由于存在形态学上的体循环右心室，HLHS患者可能比其他接受Fontan手术的成年患者面临更高的风险。由于成年HLHS患者群体持续增长，认识患者发病和死亡的常见原因变得越来越重要。患者和医疗服务提供者之间需要共同努力，以解决许多仍存在临床不确定性的领域，帮助确保患者预后和生活质量持续改善。
