Pézard P, Banus Y, Laporte J, Geslin P, Garnier H, Tadei A
Arch Mal Coeur Vaiss. 1986 Oct;79(11):1637-42.
The authors report two unusual new cases of corrected transposition of the great arteries diagnosed in patients 72 and 80 years of age respectively, presenting with symptoms of cardiac failure of recent onset. Both patients had mild or moderate regurgitation of the systemic atrio-ventricular valve, associated in one case with aortic regurgitation. This association would appear to be fortuitous although already reported in six occasions. The diagnosis was made by echocardiography and confirmed by catheterisation in one case, and by digitised intravenous angiography in the other. These cases are of interest for three reasons: They are very rare: there have been only 20 comparable cases reported in patients over 40 years of age, the eldest being 73. This is probably explained by the high frequency of associated malformations. They illustrate the decisive role of echocardiography in the diagnosis of congenital heart disease, even in the adult. They constitute a "natural experimental model" of a right ventricle submitted to systemic pressures and tend to show that, in the absence of other abnormalities giving rise to an additional pressure or volume overload, the right ventricle is capable of long term adaptation to this situation. These observations indicate a favourable long-term prognosis for children who have undergone "corrective" surgery at atrial level for simple transposition and in whom the right ventricle is required to assume the function of the systemic ventricle for the rest of their life.
作者报告了两例罕见的矫正型大动脉转位新病例，患者分别为72岁和80岁，均表现为近期发作的心力衰竭症状。两名患者均有轻度或中度的体循环房室瓣反流，其中一例合并主动脉反流。尽管已有六例报道，但这种关联似乎是偶然的。诊断通过超声心动图做出，其中一例经心导管检查证实，另一例经数字化静脉血管造影证实。这些病例之所以令人关注有三个原因：它们非常罕见：40岁以上患者中仅报道过20例类似病例，年龄最大的为73岁。这可能是由于相关畸形的发生率较高。它们说明了超声心动图在先天性心脏病诊断中的决定性作用，即使是在成人中。它们构成了一个右心室承受体循环压力的“自然实验模型”，并倾向于表明，在没有其他导致额外压力或容量负荷的异常情况下，右心室能够长期适应这种情况。这些观察结果表明，对于因单纯转位而在心房水平接受“矫正”手术且右心室在余生需承担体循环心室功能的儿童，其长期预后良好。
