Huhta J C, Danielson G K, Ritter D G, Ilstrup D M
Pediatr Cardiol. 1985;6(2):57-60. doi: 10.1007/BF02282738.
Limited information is available concerning the long-term survival of patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles (corrected transposition). The long-term follow-up of 107 patients examined at the Mayo Clinic over a 30-year period between 1951 and 1981 was reviewed. Overall survival from the date of Mayo Clinic diagnosis was 70% at five years and 64% at ten years. Associated variables were analyzed for their effect on survival including sex, age at diagnosis, presence of ventricular septal defect, pulmonary stenosis, dextrocardia, left atrioventricular valve insufficiency, and complete heart block. There was no significant difference between those with and without a ventricular septal defect (VSD). Pulmonary stenosis was protective when a VSD was present but was not a significant predictor of long-term survival. The only variable that consistently correlated with decreased survival was left atrioventricular valve insufficiency (p less than 0.04 for univariate and stepwise Cox, and p = 0.08 for multivariate analysis). A logistic model for survival after open-heart surgery failed to identify any significant variable. We conclude that the presence of left atrioventricular valve insufficiency in association with atrioventricular discordance significantly alters the long-term outcome. Atrioventricular valve replacement should be considered in such patients when insufficiency becomes hemodynamically significant.
关于房室不一致、心室动脉不一致且有两个心室（矫正性大动脉转位）患者的长期生存率，目前可用信息有限。回顾了1951年至1981年期间在梅奥诊所接受检查的107例患者的30年长期随访情况。自梅奥诊所诊断之日起的总体生存率在5年时为70%，在10年时为64%。分析了相关变量对生存率的影响，包括性别、诊断时年龄、室间隔缺损、肺动脉狭窄、右位心、左房室瓣关闭不全和完全性心脏传导阻滞。有室间隔缺损（VSD）和无室间隔缺损的患者之间无显著差异。当存在室间隔缺损时，肺动脉狭窄具有保护作用，但并非长期生存的显著预测因素。唯一与生存率降低始终相关的变量是左房室瓣关闭不全（单因素和逐步Cox分析中p小于0.04，多因素分析中p = 0.08）。心脏直视手术后生存的逻辑模型未能识别出任何显著变量。我们得出结论，左房室瓣关闭不全与房室不一致相关会显著改变长期预后。当关闭不全在血流动力学上变得显著时，此类患者应考虑进行房室瓣置换。
