改良Konno术治疗儿童左心室流出道狭窄

目的：改良Konno手术是治疗复杂左心室流出道狭窄的有效技术，本文总结该技术的应用结果。方法：回顾我院2007年1月至2018年1月应用改良Konno术治疗左心室流出道隧道样狭窄9例患者的临床资料。所有患者均有既往手术史，7例患者为二次手术，2例为三次手术。术中从左右冠窦之间主动脉瓣环下方以圆针从左室面垂直穿入右室面，用以定位室间隔切开的位置，右室双出口患者术中均全部或部分拆除原室缺补片，并予扩大，术中经食道超声评估。结果：本组男6例，女3例，中位年龄8（5-14）岁；首次手术到改良Konno手术间隔中位时间6年（4-9年）。改良Konno术中同时行主动脉瓣下纤维嵴切除3例，主动脉瓣成形2例，二尖瓣成形2例，右室流出道狭窄矫治1例。全组患者无死亡,无III度房室传导阻滞，改良Konno术前主动脉瓣环直径z值1.8±0.7，左心室流出道峰值压差86±21mmHg，术后复查峰值压差显著降低（12±4mmHg，P<0.01），主动脉瓣关闭功能良好，7例无返流，2例轻微返流。随访中位时间2.5年（3月-5年），最近一次心超提示左心室流出道峰值压差10±3mmHg，主动脉瓣功能良好，无患者需再次手术处理左心室流出道。结论：改良Konno手术有效解除左心室流出道隧道样狭窄，并保留了自体主动脉瓣功能，近中期效果满意。

Modified Konno Procedure in Pediatric Left Ventricular Outflow Tract Obstruction

Objective: Modified Konno procedure is an effective operation to relieve complex left ventricular outflow tract obstruction (LVOTO), and this study is to summarize our experiences. Methods: Nine children with tunnel-like LVOTO who operated with modified Konno procedure from Jan 2007 to Jan 2018 were retrospectively reviewed. All patients had previous cardiac surgeries. Seven patients had one previous operation, while the other two had twice. During the operation, a round needle was vertically punctured from the area below the triangle between sub-left coronary and right coronary annulus, and this was used to locate the point to incise the ventricular septum. All the previous septal patches were removed as much as possible, and the defect was enlarged. Trans-esophageal echocardiography was performed during the operation. Results: There were 6 males and 3 females, and the median age at operation was 8 (5-14) years old. The median interval between the latest and the current operation was 6 (4-9) years. The associated operations during the Konno procedure included 3 sub-aortic ridge resections, 2 aortic valvoplasty, 2 mitral valvoplasty and 1 right ventricular outflow tract obstruction relief. There was no death and no complete atrioventricular block. The z-value of aortic annulus before operation was 1.8±0.7 and the peak gradient over LVOT was 86±21mmHg. The gradient was significantly reduced to 12±4mmHg after operation before discharge (P<0.01), and the function of aortic valve was good, with no regurgitation in 7 patients and trivial regurgitation in the other two. During the follow-up of 2.5 (0.25-5) years, the latest gradient was 10±3 mmHg with well-functioned aortic valve, and no reoperation was required in term of the LVOT. Conclusions: The modified Konno procedure effectively relieved the LVOTO in our pediatric patients and preserved the native aortic valve. The short- and medium-term outcomes are good.