Vobecky S J, Williams W G, Trusler G A, Coles J G, Rebeyka I M, Smallhorn J, Burrows P, Gow R, Freedom R M
Department of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
Ann Thorac Surg. 1993 Oct;56(4):944-9; discussion 949-50. doi: 10.1016/0003-4975(93)90361-k.
The trend to perform early primary repair of tetralogy of Fallot prompted us to review our experience in the current era with the traditional protocol consisting of palliation during infancy, if required, and repair after infancy. During a 10-year period, 270 infants with tetralogy of Fallot presented aged less than 18 months. Thirteen infants (4.8%) had major noncardiac lesions that precluded definitive care for their congenital heart disease. Twenty infants (7.4%) had major associated cardiac lesions (atrioventricular septal defect or absent pulmonary valve syndrome, or both). Survival in this group was poor, with only 58% +/- 12% reaching the age of 10 years. Four of the seven deaths occurred before intracardiac repair was performed. The remaining 237 infants presented with isolated tetralogy of Fallot. Eight-nine percent +/- 2.3% survived to age 10 years. Sixty percent of these infants required palliation, and survival in these infants did not differ from that in those who never required palliation. However, 19 infants (8%) required palliation in the first month of life. In these children, survival to age 10 years was significantly lower (77%), secondary palliation was frequently required (n = 11), and a transannular patch or conduit at the time of repair (10 of 14 patients) was more likely needed than it was in children who had not undergone a palliative procedure during the neonatal period. The survival in infants with tetralogy of Fallot is unlikely to be different, regardless of whether primary repair or a staged repair is carried out. The quality of survival, including the exercise capability and absence of arrhythmias, must be assessed to determine which protocol is superior.
法洛四联症早期一期修复的趋势促使我们回顾当前时代我们在传统治疗方案下的经验，该方案包括在婴儿期（如有需要）进行姑息治疗，并在婴儿期后进行修复。在10年期间，270例年龄小于18个月的法洛四联症婴儿前来就诊。13例婴儿（4.8%）有严重的非心脏病变，无法对其先天性心脏病进行确定性治疗。20例婴儿（7.4%）有严重的相关心脏病变（房室间隔缺损或肺动脉瓣缺如综合征，或两者皆有）。该组患儿的生存率较低，只有58%±12%活到10岁。7例死亡中有4例发生在心脏内修复之前。其余237例婴儿表现为单纯性法洛四联症。89%±2.3%活到10岁。其中60%的婴儿需要姑息治疗，这些婴儿的生存率与那些从未需要姑息治疗的婴儿没有差异。然而，19例婴儿（8%）在出生后第一个月就需要姑息治疗。在这些儿童中，活到10岁的生存率显著较低（77%），经常需要二期姑息治疗（n = 11），并且在修复时（14例患者中有10例）比在新生儿期未接受姑息治疗的儿童更有可能需要跨环补片或管道。无论进行一期修复还是分期修复，法洛四联症婴儿的生存率可能没有差异。必须评估生存质量，包括运动能力和心律失常的情况，以确定哪种治疗方案更优。
