Ito T, Katogi T, Aeba R, Fujii H, Goto T, Kawada S
Department of Surgery, Keio University School of Medicine, Tokyo, Japan.
Jpn J Thorac Cardiovasc Surg. 1998 Dec;46(12):1334-8. doi: 10.1007/BF03217925.
Isolated congenital tricuspid regurgitation derived from primary dysplasia of the valvular apparatus is a rare cardiac abnormality. A 23-year-old woman was first diagnosed to have an isolated tricuspid regurgitation during infancy and was followed up at our hospital. She developed progressive cardiomegaly at the age of 22 years. The chest roentgenogram at the time of admission revealed marked cardiomegaly with a cardiothoracic ratio of 64%. Despite the severe regurgitation of the tricuspid valve, the catheter examination disclosed v and mean pressures of the right atrium of 9 mmHg and 5 mmHg, respectively. The operative findings revealed a markedly dilated tricuspid annulus of 45 mm in diameter (157% of normal) and fragile and redundant valve leaflets, anterior leaflet in particular. Anterior papillary muscle was absent without any vestige thereof. Chordae tendinae of the anterior and posterior leaflets were absent and those of the septal leaflet were attached to the ventricular septum. Each commissure was tethered to the ventricular wall by thick and short chordal tissue. The chordal abnormalities were repaired by four artificial chordae of 4-0 ePTFE suture and an annuloplasty with Carpentier-Edwards ring (36 mm) was added. She recovered uneventfully and was discharged on postoperative day 30. A follow-up echocardiography at 2 years after surgery showed excellent function and trivial regurgitation of the valve. No evidences of thrombus or calcification of the artificial chordae were detected. This experience draws us to conclude that the artificial chordal replacement is one of the useful surgical options for the repair of isolated congenital tricuspid regurgitation.
源于瓣膜装置原发性发育异常的孤立性先天性三尖瓣反流是一种罕见的心脏异常。一名23岁女性在婴儿期首次被诊断为孤立性三尖瓣反流，并在我院接受随访。她在22岁时出现进行性心脏扩大。入院时胸部X线片显示心脏明显扩大，心胸比率为64%。尽管三尖瓣存在严重反流，但心导管检查显示右心房的v波压和平均压分别为9 mmHg和5 mmHg。手术所见显示三尖瓣环明显扩张，直径达45 mm（为正常的157%），瓣膜小叶脆弱且冗长，尤其是前叶。前乳头肌缺如，无任何残留痕迹。前叶和后叶的腱索缺如，隔叶的腱索附着于室间隔。每个瓣叶联合处通过短而粗的腱索组织与心室壁相连。通过4根4-0 ePTFE缝线的人工腱索修复腱索异常，并加用Carpentier-Edwards环（36 mm）进行瓣环成形术。她恢复顺利，术后30天出院。术后2年的随访超声心动图显示瓣膜功能良好，反流轻微。未检测到人工腱索有血栓或钙化迹象。这一经验使我们得出结论，人工腱索置换是修复孤立性先天性三尖瓣反流的有效手术选择之一。
