Duro Raquel Pacheco, Moura Cláudia, Leite-Moreira Adelino
Serviço de Fisiologia, Faculdade de Medicina do Porto, Porto, Portugal.
Rev Port Cardiol. 2010 Apr;29(4):591-630.
Tetralogy of Fallot is the most frequent cyanotic congenital cardiopathy. Its physiopathology has been progressively described which has made better treatment possible. The four characteristic morphologic abnormalities are: interventricular communication, subpulmonary stenosis, biventricular origin of the aortic valve and right ventricular hypertrophy, which are the direct result of the antero-cephalad deviation of the ventricular septal outlet and hypertrophy of the septoparietal trabeculations. These anatomic abnormalities result in decreased pulmonary blood flow, leading to hypoxia and cyanosis. The main determinants of pulmonary blood flow are the source of the blood flow to the lungs, the severity and functional behaviour of the subpulmonary obstruction, the right ventricular and arterial systemic pressures and the ductus arteriosus. The mechanism of cyanotic spells is not clear. Increases in infundibular contractility, peripheral vasodilatation and right ventricular mechanoreceptor stimulation are some of the proposed mechanisms. There are two surgical strategies in newborns/children: a staged approach (with a palliative procedure followed by the complete repair) or early complete repair. There are arguments for and against each of these strategies, and the debate about the ideal treatment continues. In conclusion, the correct understanding of this cardiopathy's physiopathology is essential to improving the child's treatment. This review is particularly contemporary and relevant issue because one must always bear in mind the physiopathology of the original disease in order to correctly follow-up a new patient population: adults with surgically corrected Tetralogy of Fallot.
法洛四联症是最常见的青紫型先天性心脏病。其病理生理学已得到逐步描述，这使得更好的治疗成为可能。四个特征性形态学异常为：室间隔缺损、肺动脉瓣下狭窄、主动脉瓣双心室起源和右心室肥厚，这些是室间隔出口向前头侧偏移和隔壁小梁肥厚的直接结果。这些解剖学异常导致肺血流量减少，进而引起缺氧和青紫。肺血流量的主要决定因素包括肺血流来源、肺动脉瓣下梗阻的严重程度和功能表现、右心室和体循环动脉压力以及动脉导管。青紫发作的机制尚不清楚。提出的机制包括漏斗部收缩力增加、外周血管扩张和右心室机械感受器刺激等。对于新生儿/儿童有两种手术策略：分期手术方法（先进行姑息性手术，然后进行完全修复）或早期完全修复。这两种策略都有支持和反对的观点，关于理想治疗方法的争论仍在继续。总之，正确理解这种心脏病的病理生理学对于改善患儿的治疗至关重要。这篇综述特别关注当代相关问题，因为为了正确随访新的患者群体——接受手术矫正的法洛四联症成年患者，必须始终牢记原发病的病理生理学。
