Waldman J D, Wernly J A
University of New Mexico Health Sciences Center, Children's Hospital Heart Center, Albuquerque, USA.
Pediatr Clin North Am. 1999 Apr;46(2):385-404. doi: 10.1016/s0031-3955(05)70125-5.
Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.
在导致青紫型先天性心脏病的“五个T”中——法洛四联症、大动脉转位（TGA）、完全性肺静脉异位连接（TAPVC）、永存动脉干和三尖瓣异常（三尖瓣闭锁、狭窄及移位）——第一个和最后一个通常与肺血流量减少有关。构成法洛四联症的四个特征——右心室肥厚、室间隔缺损、主动脉骑跨及肺动脉瓣下狭窄——均继发于单一的形态发生缺陷：肺动脉圆锥发育不全。这也解释了临床表现的变异性。新生儿需要干预时，通常施行分流术。修复手术前必须明确冠状动脉解剖结构，修复手术通常在这些婴儿3个月大后进行。尽管法洛四联症修复术后的儿童并非完全“正常”，但可显著延长寿命并改善生活质量。当存在主要相关缺陷时，如房室通道缺损、细小肺动脉或侧支血管，或左心病变，预后则从极佳变为仅为良好。法洛四联症合并肺动脉瓣缺如综合征在生理上与其他法洛四联症情况不同，其主要特征是中央和肺门周围肺动脉的巨大扩张导致气道阻塞；必须进行肺动脉缩窄修复术。三尖瓣异常包括闭锁、发育不全（即室间隔完整的肺动脉闭锁）和移位（即埃布斯坦畸形）。决定这些患儿临床状况（及预后）的病理生理与三个因素有关：（1）三尖瓣状态，（2）室间隔缺损的存在及大小，（3）大动脉转位或正常相关的大动脉。几乎所有三尖瓣异常的患儿都可得到姑息治疗；修复选择包括双心室、单心室或1.5心室修复。严重肺动脉狭窄的患儿通常三尖瓣和右心室正常。球囊扩张通常是唯一必要的治疗方法。
