Nagashima M, Takeuchi Y, Gomi A, Okamura Y, Mori H
Department of Cardiovascular Surgery, Kanto Teishin Hospital, Tokyo, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1991 Jun;39(6):914-9.
Congenital corrected transposition of the great arteries (C-TGA) is frequently associated with VSD and PS. Systemic atrioventricular valve regurgitation is also sometimes present in patients with C-TGA. It is though that left-sided A-V valve regurgitation is caused by the deformities of the tricuspid valve (TV) or intolerance of TV and the right ventricle (RV) against systemic pressure load. We had a patient with C-TGA associated with Ebstein's malformation. The patient suffered from congestive heart failure due to left-sided A-V valve regurgitation. In this case, the septal and posterior leaflets were displaced below the atrioventricular annulus. The edges of all valves were thickened and partially calcified. Focal agenesis was found in the septal leaflet. There was a distinct atrialized ventricle which had a thin wall and no trabeculae. It shaped like a diverticulum of 3 cm in depth toward the diaphragm. Preoperative cardiac catheterization showed that the functional RV had an adequate size (RVEDVI 162% of normal) and ejection fraction was 53%. Left-sided valve replacement was performed using SJM 31M with preservation of TV. Left-sided atrioventricular annulus was so deformed due to the diverticulum that the prosthetic valve was sutured at the ventricular wall below the atrioventricular annulus to which TV had attached. Good clinical result was obtained after surgical treatment.
先天性矫正型大动脉转位（C-TGA）常合并室间隔缺损（VSD）和肺动脉狭窄（PS）。C-TGA患者有时也会出现体循环房室瓣反流。一般认为，左侧房室瓣反流是由三尖瓣（TV）畸形或TV及右心室（RV）对体循环压力负荷不耐受所致。我们有一位合并埃布斯坦畸形的C-TGA患者。该患者因左侧房室瓣反流而患有充血性心力衰竭。在这种情况下，隔叶和后叶移位至房室瓣环下方。所有瓣膜边缘增厚并部分钙化。隔叶存在局灶性发育不全。有一个明显的房化心室，其壁薄且无小梁。它形似一个向膈肌方向延伸3厘米深的憩室。术前心导管检查显示，功能性RV大小合适（右心室舒张末期容积指数[RVEDVI]为正常的162%），射血分数为53%。使用SJM 31M进行左侧瓣膜置换并保留TV。由于憩室，左侧房室瓣环严重变形，以致人工瓣膜缝合在TV附着的房室瓣环下方的心室壁上。手术治疗后取得了良好的临床效果。
