Abstract：
We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure. The patient was referred for surgical repair.The atrial septal defect was closed by a direct running suture. The repair of the unicuspid valve consisted in bicuspidisation by a large commissurotomy to the left anterior wall of the pulmonary artery. The neo-commissure was created with two separate patches of autologous pericardium secured to the wall of the pulmonary root. The adjustment of the effective height of the pulmonary valve leaflets was done by trimming the patches and a triangular plication of the newly created posterior leaflet. Perioperative echocardiogram showed a peak gradient of 15 mm Hg and trivial pulmonary regurgitation. The total cross-clamp time was 92 min and the bypass time 123 min with a favourable evolution after the surgery.The particularity of the case is represented by the complexity of the bicuspidisation procedure. Using this technique, a tailored approach is needed for every patient.
摘要：
我们报告了一名9岁男性患有严重的先天性肺动脉瓣狭窄的病例，该病例转介给我们的经皮瓣膜切开术中心。一入场,经胸超声心动图证实肺动脉瓣为单叶瓣,肺动脉瓣峰值/平均压差为91/53mmHg,肺环为13.8mm(-0.8Z评分).它还显示了一个扩大的RV（RV/LV比0.9）。在心脏导管插入术期间,诊断为额外的房间隔缺损（secundum），并伴有明显的左向右分流（Qp/Qs>2），不适合经皮闭合。患者被转介接受手术修复。房间隔缺损通过直接缝合闭合。单尖瓣的修复包括通过对肺动脉左前壁进行大的连缝切开术来进行双孔扩张。新连合是用固定在肺根壁上的两个单独的自体心包斑块创建的。肺动脉瓣小叶的有效高度的调整是通过修剪斑块和新创建的后小叶的三角形折叠来完成的。围手术期超声心动图显示峰值梯度为15mmHg，肺反流轻微。手术后总的交叉钳夹时间为92分钟，旁路时间为123分钟，进展良好。这种情况的特殊性表现为双叉手术的复杂性。使用这种技术，每个患者都需要量身定制的方法。
