Rodefeld Mark D, Frankel Steven H, Giridharan Guruprasad A
Section of Cardiothoracic Surgery, Indiana University School of Medicine, James Whitcomb Riley Hospital for Children, Indianapolis, IN, USA.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011;14(1):45-54. doi: 10.1053/j.pcsu.2011.01.015.
Since the Fontan/Kreutzer procedure was introduced, evolutionary clinical advances via a staged surgical reconstructive approach have markedly improved outcomes for patients with functional single ventricle. However, significant challenges remain. Early stage mortality risk seems impenetrable. Serious morbidities - construed as immutable consequences of palliation - have hardly been addressed. Late functional status is increasingly linked to pathophysiologic consequences of prior staged procedures. As more single-ventricle patients survive into adulthood, Fontan failure is emerging as an intractable problem for which there is no targeted therapy. Incremental solutions to address these ongoing problems have not had a measurable impact. Therefore, a fundamental reconsideration of the overall approach is reasonable and warranted. The ability to provide a modest pressure boost (2 to 6 mmHg) to existing blood flow at the total cavopulmonary connection can effectively restore more stable biventricular status. This would impact not only treatment of late Fontan failure, but also facilitate early surgical repair. A realistic means to provide such a pressure boost has never been apparent. Recent advances are beginning to unravel the unique challenges that must be addressed to realize this goal, with promise to open single-ventricle palliation to new therapeutic vistas.
自从引入Fontan/Kreutzer手术以来,通过分期手术重建方法取得的临床进展显著改善了功能性单心室患者的治疗效果。然而,重大挑战依然存在。早期死亡率风险似乎难以攻克。严重并发症——被视为姑息治疗不可避免的后果——几乎未得到解决。晚期功能状态越来越多地与先前分期手术的病理生理后果相关联。随着越来越多的单心室患者存活至成年,Fontan衰竭正成为一个棘手的问题,对此尚无针对性治疗方法。针对这些持续存在问题的渐进性解决方案并未产生可衡量的影响。因此,对整体方法进行根本性重新思考是合理且必要的。在全腔静脉肺动脉连接部位为现有血流提供适度压力提升(2至6 mmHg)的能力,能够有效恢复更稳定的双心室状态。这不仅会影响晚期Fontan衰竭的治疗,还将有助于早期手术修复。实现这种压力提升的现实手段一直并不明确。最近的进展开始揭示实现这一目标必须应对的独特挑战,有望为单心室姑息治疗开辟新的治疗前景。