Ponzoni Matteo, Castaldi Biagio, Padalino Massimo A
Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, 35122 Padua, Italy.
Pediatric Cardiology Unit, Department of Woman's and Child's Health, University of Padua, 35122 Padua, Italy.
Children (Basel). 2022 Sep 14;9(9):1392. doi: 10.3390/children9091392.
Current treatment paradigms for end-stage dilated cardiomyopathy (DCM) in children include heart transplantation and mechanical support devices. However, waitlist mortality, shortage of smaller donors, time-limited durability of grafts, and thrombo-hemorrhagic events affect long-term outcomes. Moreover, both these options are noncurative and cannot preserve the native heart function. Pulmonary artery banding (PAB) has been reinvented as a possible "regenerative surgery" to retrain the decompensated left ventricle in children with DCM. The rationale is to promote positive ventricular-ventricular interactions that result in recovery of left ventricular function in one out of two children, allowing transplantation delisting. Although promising, global experience with this technique is still limited, and several surgical centers are reluctant to adopt PAB since its exact biological bases remain unknown. In the present review, we summarize the clinical, functional, and molecular known and supposed working mechanisms of PAB in children with DCM. From its proven efficacy in the clinical setting, we described the macroscopic geometrical and functional changes in biventricular performance promoted by PAB. We finally speculated on the possible underlying molecular pathways recruited by PAB. An evidence-based explanation of the working mechanisms of PAB is still awaited to support wider adoption of this surgical option for pediatric heart failure.
目前儿童终末期扩张型心肌病（DCM）的治疗模式包括心脏移植和机械支持装置。然而，等待名单上的死亡率、较小供体的短缺、移植物有限的耐久性以及血栓-出血事件影响了长期预后。此外，这两种选择都无法治愈疾病，也不能保留心脏的自然功能。肺动脉环扎术（PAB）已被重新定义为一种可能的“再生手术”，用于训练DCM患儿失代偿的左心室。其原理是促进积极的心室-心室相互作用，从而使每两名患儿中有一名左心室功能恢复，得以从移植等待名单上除名。尽管前景广阔，但这项技术的全球经验仍然有限，而且由于其确切的生物学基础尚不清楚，一些外科中心不愿采用PAB。在本综述中，我们总结了PAB在DCM患儿中的临床、功能和分子方面已知及推测的作用机制。基于其在临床环境中已证实的疗效，我们描述了PAB促进的双心室性能的宏观几何和功能变化。我们最后推测了PAB可能招募的潜在分子途径。仍有待基于证据对PAB的作用机制作出解释，以支持更广泛地采用这种治疗小儿心力衰竭的手术选择。
