Nölke Lars, Azakie Anthony, Anagnostopoulos Petrous V, Alphonso Nelson, Karl Tom R
Pediatric Heart Center, UCSF Children's Hospital, San Francisco, California, USA.
Ann Thorac Surg. 2006 May;81(5):1802-7. doi: 10.1016/j.athoracsur.2005.12.001.
Congenital absence of the pulmonary valve syndrome (APVS) is a rare cardiac defect characterized by aneurysmal pulmonary arteries, hypoplastic pulmonary valve cusps, and tracheobronchial abnormalities. Absence of the pulmonary valve syndrome usually occurs in conjunction with ventricular septal defect (VSD) and right ventricular outflow tract obstruction (RVOTO). Surgical mortality rates as high as 16% to 56% have been reported. Here, we describe the surgical results using the Lecompte maneuver, reduction pulmonary arterioplasty, and a valved right ventricle to pulmonary artery (RV-PA) conduit.
Medical records and operative and echocardiography reports for all surgical APVS cases were retrospectively examined for pertinent clinical variables. A patient with left bronchial compression due to enlarged pulmonary arteries associated with totally anomalous pulmonary venous drainage (TAPVD) is included to illustrate the value of the Lecompte maneuver.
From January 2002 to December 2004, 4 children with APVS had surgery at a median age of 5 months (range, 3 months to 3.5 years). Three had malalignment VSD and RVOTO. Four had respiratory signs (cough, wheeze, tachypnea, oxygen dependence, ventilator dependence), and all 5 had evidence of tracheobronchial compression by computed tomography or magnetic resonance imaging. Repair included a Lecompte maneuver, a valved conduit with reduction pulmonary arterioplasty, and VSD closure as necessary. The TAPVD patient had repair of the anomalous veins and a Lecompte maneuver. There have been no deaths, and all patients were discharged in good condition. Follow-up is complete at a median of 24 months (range, 9 to 35). Echocardiography has shown no progressive enlargement of the pulmonary arteries.
Use of the Lecompte maneuver, reduction arterioplasty, and a valved conduit for repair of APVS provides favorable early and midterm results.
先天性肺动脉瓣缺如综合征（APVS）是一种罕见的心脏缺陷，其特征为肺动脉瘤样扩张、肺动脉瓣叶发育不全以及气管支气管异常。肺动脉瓣缺如综合征通常与室间隔缺损（VSD）和右心室流出道梗阻（RVOTO）同时出现。据报道，手术死亡率高达16%至56%。在此，我们描述了使用勒孔特手法、肺动脉缩窄成形术以及带瓣右心室至肺动脉（RV-PA）管道的手术结果。
对所有接受手术治疗的APVS病例的病历、手术记录和超声心动图报告进行回顾性检查，以获取相关临床变量。纳入一名因肺动脉扩张伴完全性肺静脉异位引流（TAPVD）导致左支气管受压的患者，以说明勒孔特手法的价值。
2002年1月至2004年12月，4例APVS患儿接受了手术，中位年龄为5个月（范围为3个月至3.5岁）。3例存在对位不良型室间隔缺损和右心室流出道梗阻。4例有呼吸症状（咳嗽、喘息、呼吸急促、氧依赖、呼吸机依赖），所有5例经计算机断层扫描或磁共振成像均显示有气管支气管受压证据。修复手术包括勒孔特手法、带瓣管道及肺动脉缩窄成形术，并根据需要关闭室间隔缺损。TAPVD患者接受了异常静脉修复和勒孔特手法。无一例死亡，所有患者均康复出院。中位随访时间为24个月（范围为9至35个月），随访完整。超声心动图显示肺动脉无进行性扩大。
使用勒孔特手法、肺动脉缩窄成形术和带瓣管道修复APVS可获得良好的早期和中期结果。
