Shimizu M, Kawaguchi T, Ogihara A, Kitazumi H, Adachi Y, Fukawa S, Maseki Y, Hasegawa N, Kikawada R
Department of Internal Medicine, Kitasato University School of Medicine.
Kokyu To Junkan. 1990 Oct;38(10):1035-40.
We report a case in a 36-year-old man of corrected transposion of the great arteries (CTGA) without any associated defects. We also review clinical characteristics and later complications of adult CTGA cases reported in Japan. The patients was referred to our hospital for further examination of the abnormal ECG found during regular medical check-up. His ECG showed left axis deviation, first degree AV block, abnormal Q waves in the left precordial leads, and disappearance of septal q waves in the left precordial leads. His exercise capacity was found to be more than 12 METS by treadmill exercise testing. His roentgenogram revealed an egg-shaped ventricle with normal CTR (45%). The following findings were obtained by two-dimensional echocardiogram and heart catheterization: 1) the functional left ventricle had the characteristics of the anatomical right ventricle, 2) The positions of the left and right atrioventricular valves were reversed, 3) Other cardiac anomalies such as VSD, pulmonary stenosis, tricuspid regurgitation (TR) were not proved. Therefore, he was diagnosed as having CTGA without any cardiac defects (SLL type). At present, with ages more than 15 years, 36 cases of CTGA without cardiac anomalies have been reported in Japan including this case. But most cases had significant TR. This case was the 7th reported case without the accompaniment of TR. Among 36 cases, TR was found in 71%, complete AV block in 20%, and congestive heart failure was noted in 45%. Prognosis of CTGA without any defects is dependent on the appearance of TR, advanced AV block, and congestive heart failure. As these later fatal complications are observed very often, it is important to detect the patient of CTGA accurately.(ABSTRACT TRUNCATED AT 250 WORDS)
我们报告一例36岁男性大动脉转位矫正术(CTGA)患者,无任何相关缺陷。我们还回顾了日本报道的成人CTGA病例的临床特征和后期并发症。该患者因在定期体检中发现异常心电图而被转诊至我院进一步检查。其心电图显示电轴左偏、一度房室传导阻滞、左胸前导联异常Q波以及左胸前导联间隔q波消失。通过跑步机运动试验发现他的运动能力超过12代谢当量。其X线胸片显示心腔呈椭圆形,心胸比率正常(45%)。通过二维超声心动图和心导管检查获得以下结果:1)功能性左心室具有解剖学右心室的特征;2)左右房室瓣位置颠倒;3)未证实存在其他心脏异常,如室间隔缺损、肺动脉狭窄、三尖瓣反流(TR)。因此,他被诊断为无任何心脏缺陷的CTGA(SLL型)。目前,在日本,包括本例在内,已报道15岁以上无心脏异常的CTGA病例36例。但大多数病例有明显的TR。本例是第7例无TR伴随的报道病例。在36例病例中,71%发现有TR,20%有完全性房室传导阻滞,45%有充血性心力衰竭。无任何缺陷的CTGA的预后取决于TR、晚期房室传导阻滞和充血性心力衰竭的出现。由于这些后期致命并发症经常被观察到,准确检测CTGA患者很重要。(摘要截断于250字)