Knott-Craig C J, Elkins R C, Lane M M, Holz J, McCue C, Ward K E
Section of Thoracic Surgery, University of Oklahoma Health Sciences Center, Oklahoma City 73190, USA.
Ann Thorac Surg. 1998 Aug;66(2):506-11. doi: 10.1016/s0003-4975(98)00493-7.
Over the past decade repair of tetralogy of Fallot (TOF) in infancy has gained favor. It is still uncertain what effect early complete repair will have on survival or late reoperation on the right ventricular outflow tract.
To assess these outcomes, we reviewed our experience (1971-1997) with 294 patients undergoing operation at one institution. Median follow-up was 10.6 years (range, 0.1 to 26 years), and was complete for 90.2% patients.
Primary complete repair was done in 199 patients (68%), and a staged repair in 62 patients (21%). Thirty-three patients had only a palliative procedure. Sixty-eight patients (23.1%) had complex pathologic processes, including pulmonary atresia in 53. Hospital mortality for primary repair was 11.1% (22/199), for staged repair was 17.7% (11/62), and for palliative procedures was 15.5% (16/103 procedures). Since 1990 mortality has been 2.1%, 11.8%, and 0% respectively (p < 0.001), despite younger age at repair (0.6+/-0.1 versus 2.1+/-0.2 years; p < 0.001). Multivariate analysis identified longer period of hypothermic circulatory arrest, pulmonary artery patch angioplasty, earlier year of operation, and closure of the foramen ovale as risk factors for hospital death. For hospital survivors 20-year survival was 98%+/-3% for TOF with pulmonary stenosis and 88%+/-9% for TOF with pulmonary atresia (p=0.09). Reintervention on the right ventricular outflow tract was needed in 14.1% (37/261) patients. Freedom from reintervention on the right ventricular outflow tract at 20 years was 86%+/-4% for TOF with pulmonary stenosis and 43%+/-16% for TOF with pulmonary atresia (p=0.001). For the subgroup TOF with pulmonary stenosis, this was 85%+/-5% after primary repair and 91%+/-8% after staged repair (not significant). At 15-year follow-up, this was 78%+/-10% for patients not older than 1 year at operation compared with 88%+/-4% for older patients (not significant).
Early mortality after primary repair of TOF has significantly improved and late survival is excellent. Primary repair in infancy does not increase risk for reintervention on the right ventricular outflow tract.
在过去十年中，婴儿期法洛四联症（TOF）修复术受到青睐。早期完全修复对生存率或右心室流出道后期再次手术有何影响仍不确定。
为评估这些结果，我们回顾了在一家机构接受手术的294例患者（1971 - 1997年）的经验。中位随访时间为10.6年（范围0.1至26年），90.2%的患者随访完整。
199例患者（68%）进行了一期完全修复，62例患者（21%）进行了分期修复。33例患者仅接受了姑息手术。68例患者（23.1%）有复杂病理过程，其中53例为肺动脉闭锁。一期修复的医院死亡率为11.1%（22/199），分期修复为17.7%（11/62），姑息手术为15.5%（16/103例手术）。自1990年以来，死亡率分别为2.1%、11.8%和0%（p < 0.001），尽管修复时年龄更小（0.6±0.1岁对2.1±0.2岁；p < 0.001）。多因素分析确定低温循环阻断时间延长、肺动脉补片血管成形术、手术年份较早和卵圆孔闭合是医院死亡的危险因素。对于医院幸存者，肺动脉狭窄型TOF的20年生存率为98%±3%，肺动脉闭锁型TOF为88%±9%（p = 0.09）。14.1%（37/261）患者需要对右心室流出道进行再次干预。肺动脉狭窄型TOF 20年无右心室流出道再次干预率为86%±4%，肺动脉闭锁型TOF为43%±16%（p = 0.001）。对于肺动脉狭窄型TOF亚组，一期修复后为85%±5%，分期修复后为91%±8%（无显著差异）。在15年随访时，手术时年龄不超过1岁的患者为78%±10%，年龄较大患者为88%±4%（无显著差异）。
TOF一期修复后的早期死亡率显著改善，后期生存率良好。婴儿期一期修复不会增加右心室流出道再次干预的风险。
