Ikeda U, Furuse M, Suzuki O, Kimura K, Sekiguchi H, Shimada K
Department of Cardiology, Jichi Medical School, Tochigi, Japan.
Chest. 1992 May;101(5):1382-5. doi: 10.1378/chest.101.5.1382.
Corrected transposition of the great arteries is a rare condition, and few patients with this abnormality survive past 50 years of age because of associated congenital defects or the subsequent development of atrioventricular valvular insufficiency or heart block or both. We describe four men with uncomplicated C-TGA. Our patients are of interest for the following reasons: (a) their condition is very rare; (b) the diagnosis of C-TGA traditionally has been verified through invasive cardiac catheterization procedures; however, in our latest two patients, recently developed noninvasive diagnostic techniques played the decisive role in the diagnosis of C-TGA; (c) in these modalities, they presented as a "natural experimental model" that the right ventricle submitted to a high systemic pressure load is capable of increasing muscle mass over long-term adaptation. Our four patients illustrate that patients with C-TGA, even with the associated cardiac anomalies, may live a normal life span with proper management.
矫正型大动脉转位是一种罕见的疾病，由于合并先天性缺陷或随后出现房室瓣膜功能不全或心脏传导阻滞或两者皆有，很少有患有这种异常的患者能活过50岁。我们描述了4例无并发症的矫正型大动脉转位男性患者。我们的患者之所以令人感兴趣，原因如下：(a) 他们的病情非常罕见；(b) 矫正型大动脉转位的诊断传统上是通过侵入性心脏导管检查程序来证实的；然而，在我们最近的两名患者中，最近开发的非侵入性诊断技术在矫正型大动脉转位的诊断中起了决定性作用；(c) 在这些情况下，他们呈现为一个“自然实验模型”，即承受高体循环压力负荷的右心室能够通过长期适应增加肌肉质量。我们的4例患者表明，矫正型大动脉转位患者即使合并心脏异常，通过适当的管理也可能拥有正常的寿命。
