Department of Physiology, University of Toronto, Toronto, Ontario, Canada.
Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Am J Physiol Heart Circ Physiol. 2022 Jan 1;322(1):H1-H7. doi: 10.1152/ajpheart.00451.2021. Epub 2021 Oct 15.
Despite recent advances in pediatric diagnosis and surgical intervention, mortality and morbidity continue to be a prevalent issue in both tetralogy of Fallot (ToF) and hypoplastic left heart syndrome (HLHS). Therefore, novel approaches to studying both of these conditions are warranted. Investigating cardiac anatomical features of different species in the animal kingdom that are similar to the defects and complications present in ToF and HLHS (as well as others) could serve as a new avenue for improving the management of congenital heart diseases (CHD). This review reveals that although some structures found in HLHS and ToF are pathological, similar structures can be found in diving mammals and reptiles that are adaptive. Pathological aortic dilation in CHD resembles the aortic bulb present in diving mammals, but the latter is more elastic and distensible compared with the former. The unrepaired HLHS heart resembles the univentricular heart of non-crocodilian reptiles. Right ventricle hypertrophy is pathological in HLHS and ToF, but in contrast, adaptive in crocodilians and diving mammals. Lastly, the increased pulmonary resistance due to pulmonary stenosis in ToF is comparable with increased pulmonary resistance in crocodilians due to the presence of an active valve proximal to the pulmonary valve. Some of these anatomical structures could potentially be adapted for palliative surgery in children with HLHS or ToF. Moreover, further investigating the underlying molecular signals responsible for the adaptive tissue responses seen in other species may also be useful for developing novel strategies for preventing some of the complications that occur after surgical repair in both of these congenital heart diseases.
尽管儿科诊断和外科干预技术最近取得了进展，但法洛四联症（ToF）和左心发育不全综合征（HLHS）的死亡率和发病率仍然是一个普遍存在的问题。因此，有必要研究这些疾病的新方法。研究动物王国中与 ToF 和 HLHS（以及其他疾病）中存在的缺陷和并发症相似的不同物种的心脏解剖特征，可以为改善先天性心脏病（CHD）的管理提供新途径。这篇综述表明，尽管 HLHS 和 ToF 中存在一些结构是病理性的，但在潜水哺乳动物和爬行动物中也可以发现类似的适应性结构。CHD 中的病理性主动脉扩张类似于潜水哺乳动物的主动脉球，但后者比前者更具弹性和可扩展性。未修复的 HLHS 心脏类似于非鳄目爬行动物的单心室心脏。HLHS 和 ToF 中的右心室肥大是病理性的，但与之相反，在鳄目动物和潜水哺乳动物中是适应性的。最后，由于 ToF 中的肺动脉瓣狭窄导致的肺阻力增加与由于肺动脉瓣近端存在活动瓣膜而导致的鳄目动物中的肺阻力增加相当。这些解剖结构中的一些可能会被用于 HLHS 或 ToF 患儿的姑息性手术。此外，进一步研究其他物种中适应性组织反应的潜在分子信号，也可能有助于开发预防这两种先天性心脏病手术后发生的一些并发症的新策略。
