Jacob J L
Instituto de Moléstias Cardiovasculares de São José do Rio Preto, São José do Rio Preto, SP, Brazil.
Arq Bras Cardiol. 2001 Oct;77(4):355-60. doi: 10.1590/s0066-782x2001001000005.
Few patients with corrected transposition of the great arteries survive past 50 years of age because of the association with congenital defects, development of total atrioventricular block, and right ventricular dysfunction. We report the case of a male patient with dextrocardia in situs solitus and corrected transposition of the great arteries associated with a wide atrial septal defect and severe pulmonary valvar and subvalvar stenoses. The patient also developed a large aneurysm on the pulmonary artery, total atrioventricular block diagnosed 8 years earlier, symptoms of dysfunction of the systemic ventricle in the previous 2 years, insufficiency of the left atrioventricular valve, and aortic regurgitation. Despite all these associated anomalies, the patient developed class III cardiac decompensation only at the age of 68 years, which makes this case a rarity. The patient was clinically treated, and was discharged from the hospital in good condition.
由于合并先天性缺陷、完全性房室传导阻滞的发生以及右心室功能障碍，很少有矫正型大动脉转位患者能活过50岁。我们报告一例男性患者，心脏右位，位于正常位置，患有矫正型大动脉转位，合并巨大房间隔缺损以及严重的肺动脉瓣和瓣下狭窄。该患者还出现了肺动脉上的一个大动脉瘤，8年前诊断为完全性房室传导阻滞，前两年出现体循环心室功能障碍症状，左房室瓣关闭不全，以及主动脉瓣反流。尽管存在所有这些相关异常，但该患者直到68岁才出现Ⅲ级心脏失代偿，这使得该病例非常罕见。该患者接受了临床治疗，出院时情况良好。
