Bevilacqua M, Sanders S P, Van Praagh S, Colan S D, Parness I
Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115.
J Am Coll Cardiol. 1991 Aug;18(2):559-68. doi: 10.1016/0735-1097(91)90615-g.
The echocardiographic anatomy of double-inlet single left ventricle was studied in 57 patients, aged 1 day to 27 years (mean 6 years); the variables examined included morphology, size and function of the atrioventricular (AV) valves and ventricular septal defect and their relation to pulmonary stenosis, aortic stenosis and aortic arch obstruction. The visceroatrial situs was solitus and the heart was in the left side of the chest in all 57 patients. A d-loop ventricle was present in 21 patients and an l-loop ventricle in 36. The great arteries were normally related (Holmes heart) in 8 patients and transposed in 49. In all hearts, the right AV valve was anterior to the left AV valve. In 53 patients, the tricuspid valve (right valve in d-loop and left valve in l-loop) was closer to and had attachments on the septum. The tricuspid valve straddled the outflow chamber in eight patients. No significant difference was noted in the mean AV valve diameter when comparing mitral and tricuspid valves within the same group or between the groups with a d- or l-loop ventricle. The right AV valve diameter had a significant direct correlation with the aortic valve diameter and the size of the ventricular septal defect regardless of ventricular loop. Both AV valves were functionally normal in 34 patients. Among patients with AV valve dysfunction, the tricuspid valve tended to be stenotic in patients with an l-loop ventricle and regurgitant in patients with a d-loop ventricle. Mitral valve dysfunction was uncommon. The ventricular septal defect (46 patients) was separated from the semilunar valves in 24 patients (muscular defect) and adjacent to the anterior semilunar valve as a result of hypoplasia or malalignment, or both, of the infundibular septum (subaortic defect) in 19 patients. Multiple defects were present in three patients. The defect was unrestrictive in 26 patients, restrictive in 23 and could not be evaluated in 8. Pulmonary artery banding had been performed in 8 of the 26 patients with an unrestrictive defect and in 10 of the 23 patients with a restrictive defect. Only 4 of 19 subaortic defects compared with 16 of 24 muscular defects were restrictive. The size of the defect was significantly correlated with the measured pressure gradient. Among patients with transposition, only 2 of 13 with pulmonary stenosis had a restrictive ventricular septal defect compared with 15 of 30 without pulmonary stenosis. In patients with transposition, the defect size was significantly smaller when coarctation was present.
对57例年龄在1天至27岁（平均6岁）的双入口单左心室患者进行了超声心动图解剖学研究；所检查的变量包括房室（AV）瓣的形态、大小和功能、室间隔缺损及其与肺动脉狭窄、主动脉狭窄和主动脉弓梗阻的关系。57例患者的内脏心房位置均为正位，心脏位于胸腔左侧。21例患者为d袢心室，36例为l袢心室。8例患者的大动脉关系正常（霍姆斯心脏），49例发生转位。在所有心脏中，右房室瓣位于左房室瓣前方。53例患者中，三尖瓣（d袢中的右瓣和l袢中的左瓣）更靠近间隔并附着于其上。8例患者的三尖瓣跨越流出腔。在同一组内或d袢或l袢心室组之间比较二尖瓣和三尖瓣时，平均房室瓣直径无显著差异。无论心室袢如何，右房室瓣直径与主动脉瓣直径和室间隔缺损大小均呈显著正相关。34例患者的两个房室瓣功能均正常。在房室瓣功能障碍的患者中，l袢心室患者的三尖瓣倾向于狭窄，d袢心室患者的三尖瓣倾向于反流。二尖瓣功能障碍不常见。室间隔缺损（46例患者）中，24例患者的缺损与半月瓣分离（肌部缺损），19例患者由于漏斗间隔发育不全或排列不齐或两者兼有，缺损与前半月瓣相邻（主动脉下缺损）。3例患者存在多个缺损。26例患者的缺损为非限制性，23例为限制性，8例无法评估。26例非限制性缺损患者中有8例、23例限制性缺损患者中有10例进行了肺动脉环扎术。19例主动脉下缺损中只有4例与24例肌部缺损中的16例为限制性缺损。缺损大小与测得的压力阶差显著相关。在转位患者中，13例有肺动脉狭窄的患者中只有2例有限制性室间隔缺损，而30例无肺动脉狭窄的患者中有15例。在转位患者中，存在缩窄时缺损大小明显较小。
