Jansen C, Hruda J, Rammeloo L, Ottenkamp J, Hazekamp M G
Department of Pediatrics, VU Medical Center, Center for Congenital Anomalies of the Heart Amsterdam/Leiden, Amsterdam, The Netherlands.
Pediatr Cardiol. 2006 Sep-Oct;27(5):552-6. doi: 10.1007/s00246-006-1297-0. Epub 2006 Aug 23.
An aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate semilunar valves. In order to increase our understanding about the surgical management of this rare lesion and its long-term results, we describe our experience over a 37-year period. Between 1968 and 2005, 18 patients were diagnosed with APW. Seventeen underwent surgical correction. Age at operation ranged from 22 days to 22 years (median, 0.20 years). Follow-up ranged from 2 weeks to 28.6 years (median, 11.0 years). Surgical closure was achieved using a single patch in 7 patients (41.2%) double patch in 4 (23.5%), primary closure in 3 (17.6%), clip in 2 (11.8%), and ligation in 1 (5.9%). Complex APW was present in 8 patients (44.4%). One patient was treated nonsurgically. There were no early or late deaths after surgery. Both primary closure and patch closure gave excellent long-term results. Sporadic postoperative complications were only associated with complex lesions. One patient who was treated conservatively died (of pulmonary hypertension) 21 years after diagnosis. Repair of APW is ideally performed in the first months of life, before irreversible PHT has developed. Various surgical repair techniques in this series of patients gave excellent short-term and long-term results, without significant hemodynamic sequelae.
主肺动脉窗(APW)是指在存在两个独立半月瓣的情况下升主动脉与肺动脉干之间的交通。为了增进我们对这种罕见病变的外科治疗及其长期结果的了解,我们描述了我们37年间的经验。1968年至2005年间,18例患者被诊断为APW。17例接受了手术矫正。手术年龄从22天至22岁(中位数为0.20岁)。随访时间从2周至28.6年(中位数为11.0年)。7例患者(41.2%)采用单片修补实现手术闭合,4例(23.5%)采用双片修补,3例(17.6%)采用一期闭合,2例(11.8%)采用夹子夹闭,1例(5.9%)采用结扎。8例患者(44.4%)存在复杂APW。1例患者接受非手术治疗。术后无早期或晚期死亡。一期闭合和补片闭合均取得了优异的长期效果。偶发的术后并发症仅与复杂病变相关。1例接受保守治疗的患者在诊断后21年死于(肺动脉高压)。APW的修复最好在出生后的头几个月进行,即在不可逆的肺动脉高压(PHT)发展之前。本系列患者采用的各种手术修复技术均取得了优异的短期和长期效果,且无明显的血流动力学后遗症。