Rychik J, Jacobs M L, Norwood W I
Division of Cardiology, Children's Hospital of Philadelphia, PA 19104.
Circulation. 1994 Nov;90(5 Pt 2):II13-9.
Obstruction to left ventricular outflow can be seen after surgical repair of congenital heart disease in which a ventricular septal defect (VSD) is closed by means of a baffle to the systemic great artery arising from the right ventricle (Rastelli operation, intraventricular repair, for conotruncal anomaly). We investigated the hypothesis that obligatory volumetric changes that occur after this operation lead to acute alterations in ventricular geometry and VSD size, resulting in subsequent subaortic stenosis in patients who were thought before operation to have a large, nonrestrictive VSD orifice.
Preoperative and postoperative echocardiograms and medical records of 24 patients with conotruncal anomaly who underwent conventional Rastelli operation or intraventricular repair in which the VSD was used as part of a new left ventricular outflow were reviewed. Eleven patients had transposition of the great arteries with pulmonic stenosis, 7 had double-outlet right ventricle, and 6 had subaortic atresia or stenosis with a normal-size left ventricle and underwent Norwood's palliation in infancy. All had large, nonrestrictive VSDs at preoperative cardiac catheterization. The mean age at the time of surgery was 32 +/- 24 months. The following measurements were made from two-dimensional echocardiographic images obtained before and 5 +/- 4 days after surgery from the subcostal views at end diastole: (1) VSD diameter; (2) short-axis left ventricular internal diameter (LVID); (3) left ventricular posterior wall thickness (LVPW); and (4) systemic great artery diameter (arising from the right ventricle). VSD diameter diminished significantly after surgery (11.6 +/- 3.6 versus 10.1 +/- 3.7 mm, P < .0001), as did LVID (34.9 +/- 5.0 versus 31.7 +/- 5.1 mm, P < .001). LVPW thickness increased significantly (5.7 +/- 1.0 versus 6.7 +/- 1.1 mm, P < .0001), while great artery diameter was unchanged (16.2 +/- 4.0 versus 16.7 +/- 3.8 mm, P = NS). Percent change in VSD dimension correlated with percent change in LVPW/LVIDD ratio (degree of ventricular "contraction"). Nine patients subsequently developed subaortic obstruction at the VSD orifice level and had a greater degree of early diminution in VSD size (21 +/- 8% versus 10 +/- 8%, P < .002) as well as postoperative change in LVPW/LVID ratio (0.24 +/- 0.04 versus 0.20 +/- 0.02, P < .002) than those who did not develop subsequent subaortic obstruction.
The left ventricle undergoes geometric change after Rastelli operation or intraventricular repair, surgeries in which the VSD is used as the new left ventricular outflow. These changes are manifested as increased wall thickness, decreased cavity dimensions, and a decrease in VSD size. Patients who subsequently develop left ventricular outflow obstruction have the greatest degree of ventricular contraction and VSD diminution early after surgery.
在先天性心脏病手术修复后可出现左心室流出道梗阻,此类先天性心脏病通过将室间隔缺损(VSD)用一块挡板封堵至发自右心室的体循环大动脉(Rastelli手术、心室内修复术,用于圆锥干畸形)。我们研究了这样一种假说,即该手术后发生的必然容量变化会导致心室几何形状和VSD大小的急性改变,从而在术前被认为有大的、非限制性VSD孔的患者中导致随后的主动脉下狭窄。
回顾了24例接受传统Rastelli手术或心室内修复术(其中VSD被用作新的左心室流出道的一部分)的圆锥干畸形患者的术前和术后超声心动图及病历。11例患者患有大动脉转位合并肺动脉狭窄,7例患有右心室双出口,6例患有主动脉下闭锁或狭窄且左心室大小正常,并在婴儿期接受了诺伍德姑息手术。所有患者在术前心导管检查时均有大的、非限制性VSD。手术时的平均年龄为32±24个月。从舒张末期肋下视图获取的二维超声心动图图像中,在术前和术后5±4天进行了以下测量:(1)VSD直径;(2)左心室内径短轴(LVID);(3)左心室后壁厚度(LVPW);(4)体循环大动脉直径(发自右心室)。术后VSD直径显著减小(11.6±3.6对10.1±3.7mm,P<.0001),LVID也显著减小(34.9±5.0对31.7±5.1mm,P<.001)。LVPW厚度显著增加(5.7±1.0对6.7±1.1mm,P<.0001),而大动脉直径无变化(16.2±4.0对16.7±3.8mm,P=无显著性差异)。VSD尺寸的百分比变化与LVPW/LVIDD比值(心室“收缩”程度)的百分比变化相关。9例患者随后在VSD孔水平出现主动脉下梗阻,与未发生随后主动脉下梗阻的患者相比,其VSD大小的早期减小程度更大(21±8%对10±8%,P<.002),以及术后LVPW/LVID比值的变化更大(0.24±0.04对0.20±0.02,P<.002)。
在Rastelli手术或心室内修复术后,左心室会发生几何形状改变,在这些手术中VSD被用作新的左心室流出道。这些变化表现为壁厚增加、腔尺寸减小和VSD大小减小。随后发生左心室流出道梗阻的患者在术后早期心室收缩和VSD减小程度最大。