Musewe N N, Robertson M A, Benson L N, Smallhorn J F, Burrows P E, Freedom R M, Moes C A, Rowe R D
Br Heart J. 1987 Apr;57(4):364-70. doi: 10.1136/hrt.57.4.364.
The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation. Balloon dilatation was performed by conventional techniques. In one patient, who had balloon dilatation in the operating room before surgical valvectomy, the diameter of the valve orifice increased from 3 mm to 10 mm. Inspection showed a tear along the anterior commissure. The mean (SD) pressure gradients between the right ventricle and pulmonary artery before and immediately after dilatation in five patients were not significantly different (58 (28) and 47 (12) mm Hg) respectively. There was no overall significant change in the degree of stenosis when four of these patients were examined by Doppler echocardiography six months after operation (44 (17) mm Hg), although one patient (case 5) did show a significant reduction in gradient. This patient had angiographic and echocardiographic features of dysplasia and commissural fusion. Several echographic features were common to all patients and distinguished them from cases of typical pulmonary valve stenosis. These were: pronounced thickening of leaflets; leaflet immobility in diastole and systole; no dilatation of the sinuses of Valsalva in diastole, and supra-annular narrowing. These poor results of balloon dilatation suggest that commissural fusion is not an important mechanism for causing stenosis in the dysplastic pulmonary valve. When dysplasia of the pulmonary valve is identified clinically and echocardiographically, balloon dilatation is unlikely to improve haemodynamic function; it should be attempted if commissural fusion is present.
在为期两年的时间里，从38例因肺动脉瓣狭窄而被选来进行球囊扩张术的患者中，识别出7例经血管造影证实为发育异常的患者（5例女性，平均年龄2岁），评估使用球囊扩张术缓解肺动脉瓣发育异常所致狭窄的可行性。3例患者的临床特征符合努南综合征。在所有患者中，于心脏导管插入术前通过横截面超声心动图和多普勒超声心动图评估瓣膜两端的压力阶差。采用传统技术进行球囊扩张术。1例患者在手术室进行手术瓣膜切除术前接受了球囊扩张术，瓣膜口直径从3毫米增加到10毫米。检查显示沿前联合处有撕裂。5例患者扩张前和扩张后即刻右心室与肺动脉之间的平均（标准差）压力阶差分别为58（28）和47（12）毫米汞柱，差异无统计学意义。术后6个月，当对其中4例患者进行多普勒超声心动图检查时，狭窄程度总体上无显著变化（44（17）毫米汞柱），不过1例患者（病例5）的压力阶差确实显著降低。该患者具有发育异常和联合融合的血管造影及超声心动图特征。所有患者均有一些超声心动图特征，这些特征将他们与典型肺动脉瓣狭窄病例区分开来。这些特征包括：瓣叶明显增厚；舒张期和收缩期瓣叶活动度消失；舒张期瓦尔萨尔瓦窦无扩张，以及瓣环上狭窄。球囊扩张术的这些不良结果表明，联合融合并非发育异常的肺动脉瓣狭窄形成的重要机制。当临床上和超声心动图上识别出肺动脉瓣发育异常时，球囊扩张术不太可能改善血流动力学功能；如果存在联合融合则应尝试进行球囊扩张术。
