Adult Congenital Heart Disease Unit, La Paz University Hospital, Madrid, Spain.
Am J Cardiol. 2012 Dec 1;110(11):1687-91. doi: 10.1016/j.amjcard.2012.07.039. Epub 2012 Aug 28.
The long-term outcome of patients with congenitally corrected transposition of the great arteries is mainly determined by progressive morphologically tricuspid valve regurgitation, heart block, atrial arrhythmias, and/or systemic ventricular dysfunction. Situs abnormalities have been reported in ≤34% of cases, but whether clinical differences exist between a situs inversus and situs solitus arrangement has not yet been studied. The clinical records of 38 adults with congenitally corrected transposition of the great arteries (mean age 40 ± 15 years) followed for a mean period of 7.4 years were reviewed. Of these 38 patients, 8 presented with situs inversus and 30 with situs solitus. No significant differences were found between the 2 groups in age, gender, ventricular septal defect, pulmonary tract stenosis, previous surgical repair, or duration of follow-up. However, none of the patients with situs inversus presented with an Ebstein-like anomaly of the morphologically tricuspid valve and none developed nonoperative-related complete atrioventricular block compared to 15 (50%; p = 0.013) and 11 (42%; p = 0.032) of the patients with situs solitus, respectively. At follow-up, 2 patients with situs inversus (25%) presented with sustained atrial arrhythmia, severe tricuspid regurgitation, or severe systemic right ventricular systolic dysfunction compared to 22 (73%) of 30 those with situs solitus (p = 0.034). No patient with situs inversus presented with cardiac death or severe heart failure compared to 12 (40%) of 30 with situs solitus (p = 0.038). In conclusion, Ebstein-like anomaly or spontaneous complete atrioventricular block are rare in patients with congenitally corrected transposition of the great arteries with situs inversus, and late complications are uncommon. The long-term outcome of patients with situs inversus was significantly better than that for patients with situs solitus.
患者患有先天性矫正性大动脉转位的长期预后主要取决于进行性形态三尖瓣反流、心脏传导阻滞、房性心律失常和/或系统性心室功能障碍。报道称，≤34%的病例存在内脏位置异常，但 situs 反转和 situs solitus 排列之间是否存在临床差异尚未研究。回顾了 38 例先天性矫正性大动脉转位成人（平均年龄 40 ± 15 岁）的临床记录，平均随访时间为 7.4 年。在这 38 例患者中，8 例存在 situs 反转，30 例存在 situs solitus。两组在年龄、性别、室间隔缺损、肺动脉狭窄、既往手术修复或随访时间方面无显著差异。然而，与 situs solitus 组的 15 例（50%；p = 0.013）和 11 例（42%；p = 0.032）相比，没有 situs 反转的患者出现形态三尖瓣的 Ebstein 样异常，也没有出现非手术相关的完全性房室传导阻滞。随访时，与 situs solitus 组的 22 例（73%）相比，2 例 situs 反转患者（25%）出现持续性房性心律失常、严重三尖瓣反流或严重系统性右心室收缩功能障碍（p = 0.034）。与 situs solitus 组的 30 例患者相比，没有 situs 反转患者出现心脏性死亡或严重心力衰竭（p = 0.038）。结论，先天性矫正性大动脉转位伴 situs 反转患者中 Ebstein 样异常或自发性完全性房室传导阻滞罕见，晚期并发症少见。Situs 反转患者的长期预后明显优于 situs solitus 患者。
