Murphy J G, Gersh B J, Mair D D, Fuster V, McGoon M D, Ilstrup D M, McGoon D C, Kirklin J W, Danielson G K
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minn. 55905.
N Engl J Med. 1993 Aug 26;329(9):593-9. doi: 10.1056/NEJM199308263290901.
Although corrective surgery for tetralogy of Fallot has been available for more than 30 years, the occurrence of late sudden death in patients in whom surgery was apparently successful remains worrisome.
We studied long-term survival among 163 patients who survived 30 days after complete repair of tetralogy of Fallot, examining follow-up hospital records and death certificates when relevant.
The overall 32-year actuarial survival rate among all patients who survived surgery was 86 percent, as compared with an expected rate of 96 percent in a control population matched for age and sex (P < 0.01). Thirty-year actuarial survival rates were calculated for the patient subgroups. The survival rates among patients less than 5 years old, 5 to 7 years old, and 8 to 11 years old were 90, 93, and 91 percent, respectively--slightly less than the expected rates (P < 0.001, P = 0.06, and P = 0.02). Among patients 12 years old or older at the time of surgery, the survival rate was 76 percent, as compared with an expected rate of 93 percent (P < 0.001). The performance of a palliative Blalock-Taussig shunt procedure before repair, unlike the performance of a Waterston or Potts shunt procedure, was not associated with reduced long-term survival, nor was the need for a trans-annular patch at the time of surgery. Independent predictors of long-term survival were older age at operation (P = 0.02) and a higher ratio of right ventricular to left ventricular systolic pressure after surgery (P = 0.008). Late sudden death from cardiac causes occurred in 10 patients during the 32-year period.
Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.
尽管法洛四联症的矫正手术已开展30多年，但手术看似成功的患者出现晚期猝死的情况仍令人担忧。
我们研究了163例法洛四联症完全修复术后存活30天的患者的长期生存情况，必要时查阅随访医院记录和死亡证明。
所有手术存活患者的32年精算生存率为86%，而年龄和性别匹配的对照人群的预期生存率为96%（P<0.01）。计算了患者亚组的30年精算生存率。5岁以下、5至7岁和8至11岁患者的生存率分别为90%、93%和91%，略低于预期生存率（P<0.001、P=0.06和P=0.02）。手术时年龄在12岁及以上的患者，生存率为76%，而预期生存率为93%（P<0.001）。与Waterston或Potts分流术不同，修复前进行姑息性Blalock-Taussig分流术与长期生存率降低无关，手术时使用跨环补片也与长期生存率降低无关。长期生存的独立预测因素是手术时年龄较大（P=0.02）和术后右心室与左心室收缩压比值较高（P=0.008）。在32年期间，10例患者因心脏原因发生晚期猝死。
在接受法洛四联症手术修复的患者中，术后长期生存率很高，但仍低于普通人群。晚期猝死风险较小。
