Seddio Francesco, Migliazza Lucia, Borghi Adele, Crupi Giancarlo
Centre for the Diagnosis and Treatment of Congenital Heart Defects, Ospedali Riuniti di Bergamo, Bergamo, Italy.
J Cardiovasc Med (Hagerstown). 2007 Feb;8(2):119-22. doi: 10.2459/01.JCM.0000260214.27450.c9.
Primary repair is the treatment of choice in patients with tetralogy of Fallot. The timing of repair, however, remains controversial, and an initial palliative procedure might be considered a valuable option in the early management of symptomatic young infants and in those with either unfavourable anatomy, major associated lesions or chromosomal abnormalities with a poor life expectancy.
We reviewed the management of 100 consecutive patients with tetralogy of Fallot who were operated upon at our department during an 8-year period from June 1995 to March 2003. The rationale for the choice of the initial management and the outcome in terms of morbidity and mortality in patients who underwent primary repair was compared to that observed in patients who had had a two-stage repair.
Age less than 3 months, the presence of either an unfavourable anatomy or major associated defects and genetic disorders with poor life expectancy were the indications for an initial palliation, which was carried out in 31 patients. There were no hospital deaths, and 28 of these patients underwent later repair with one hospital death (3.5%). Two patients with severe chromosomal abnormalities died at home and the remainder required a further palliation because of severely hypoplastic pulmonary arteries. Primary repair was carried out in 69 patients with one hospital death (1.4%). A transannular patch, which was used in 80% of our patients, was not an incremental risk factor for death regardless of the type of repair. Eight patients were reoperated on because of either residual right ventricular outflow tract obstruction with (four patients) or without (one patient) residual ventricular septal defect or isolated residual ventricular septal defect (three patients). All reoperations occurred in patients undergoing primary repair.
The outcome of patients undergoing repair of tetralogy of Fallot is not influenced in terms of either mortality or morbidity by an initial palliative procedure.
一期修复是法洛四联症患者的首选治疗方法。然而，修复的时机仍存在争议，对于有症状的小婴儿以及解剖结构不佳、存在主要相关病变或染色体异常且预期寿命较短的患者，初始姑息性手术可能被视为早期管理中的一个有价值的选择。
我们回顾了1995年6月至2003年3月这8年间在我科接受手术的100例连续法洛四联症患者的治疗情况。将接受一期修复患者的初始治疗选择依据以及发病率和死亡率方面的结果，与接受两期修复患者的情况进行了比较。
年龄小于3个月、解剖结构不佳或存在主要相关缺陷以及预期寿命较短的遗传疾病是初始姑息治疗的指征，31例患者接受了初始姑息治疗。无住院死亡病例，其中28例患者后来接受了修复手术，有1例住院死亡（3.5%）。2例严重染色体异常患者在家中死亡，其余患者因肺动脉严重发育不良需要进一步姑息治疗。69例患者接受了一期修复，有1例住院死亡（1.4%）。我们80%的患者使用了跨环补片，无论修复类型如何，跨环补片都不是死亡的额外危险因素。8例患者因残余右心室流出道梗阻（4例）伴或不伴残余室间隔缺损（1例）或孤立性残余室间隔缺损（3例）而接受再次手术。所有再次手术均发生在接受一期修复的患者中。
初始姑息性手术在死亡率或发病率方面均不影响法洛四联症患者修复后的结果。
