Dallaire Frederic, Wald Rachel M, Marelli Ariane
Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine and Heath Sciences, University of Sherbrooke, 3001, 12e Avenue Nord, Sherbrooke, QC, J1H 5N4, Canada.
Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, Canada.
Pediatr Cardiol. 2017 Aug;38(6):1097-1105. doi: 10.1007/s00246-017-1656-z. Epub 2017 Jun 16.
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. As a result of the surgical strategies employed at the time of initial repair, chronic pulmonary regurgitation (PR) is prevalent in this population. Despite sustained research efforts, patient selection and timing of pulmonary valve replacement (PVR) to address PR in young asymptomatic patients with repaired tetralogy of Fallot (rToF) remain a fundamental but as yet unanswered question in the field of congenital heart disease. The ability of the heart to compensate for the chronic volume overload imposed by PR is critical in the evaluation of the risks and benefits of PVR. The difficulty in clarifying the functional impact of PR on the cardiovascular capacity may be in part responsible for the uncertainty surrounding the timing of PVR. Cardiopulmonary exercise testing (CPET) may be used to assess abnormal cardiovascular response to increased physiologic demands. However, its use as a tool for risk stratification in asymptomatic adolescents and young adults with rToF is still ill-defined. In this paper, we review the role of CPET as a potentially valuable adjunct to current risk stratification strategies with a focus on asymptomatic rToF adolescents and young adults being considered for PVR. The role of maximal and submaximal exercise measurements to identify young patients with a decreased or borderline low peak VO resulting from impaired ventricular function is explored. Current knowledge gaps and research perspectives are highlighted.
法洛四联症是最常见的青紫型先天性心脏病。由于初次修复时所采用的手术策略，慢性肺动脉反流（PR）在这一人群中很普遍。尽管进行了持续的研究，但对于患有法洛四联症修复术后（rToF）的年轻无症状患者，选择合适的患者以及确定肺动脉瓣置换（PVR）的时机以解决PR问题，仍然是先天性心脏病领域一个基本但尚未得到解答的问题。心脏对PR所导致的慢性容量超负荷进行代偿的能力，在评估PVR的风险和益处时至关重要。难以阐明PR对心血管功能的影响，可能部分导致了围绕PVR时机的不确定性。心肺运动试验（CPET）可用于评估心血管系统对增加的生理需求的异常反应。然而，其作为无症状的rToF青少年和年轻成人风险分层工具的应用仍不明确。在本文中，我们回顾了CPET作为当前风险分层策略潜在有价值辅助手段的作用，重点关注考虑进行PVR的无症状rToF青少年和年轻成人。探讨了最大和次最大运动测量在识别因心室功能受损导致峰值VO₂降低或临界低值的年轻患者中的作用。强调了当前的知识空白和研究前景。
