Rijnberg Friso M, Sojak Vladimir, Blom Nico A, Hazekamp Mark G
1 Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, the Netherlands.
2 Department of Pediatric Cardiology, Leiden University Medical Center, Leiden and Academic Medical Center, Amsterdam, the Netherlands.
World J Pediatr Congenit Heart Surg. 2018 Nov;9(6):638-644. doi: 10.1177/2150135118793087. Epub 2018 Aug 22.
Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has generally been replaced by a Damus-Kaye-Stansel or Norwood procedure due to concerns of iatrogenic heart block, reobstruction, or ventricular dysfunction. Studies reporting long-term outcome after the direct approach are limited. The aim of our study was to describe and analyze our experience with direct relief of subaortic stenosis in single ventricle patients.
Demographic data, characteristics, and pre-operative, operative and outcome details were collected for children undergoing direct relief of subaortic stenosis between 1989 and 2016.
Twenty-three patients (median age: 7.4 months, range: 10 days to 5.5 years) underwent direct relief of subaortic stenosis. Complete follow-up was available for all patients (median: 15.6 years, range: 34 days to 26.3 years). Seven (30%) patients had recurrence of subaortic stenosis. One (4%) patient developed complete heart block and one patient developed moderate ventricular dysfunction. Five (50%) patients developed a (pseudo)aneurysm at site of the patch and ventriculotomy. There were two perioperative deaths. Eighty-six percent of patients underwent a successful Fontan procedure.
Direct relief of subaortic stenosis is associated with a substantial risk of reobstruction and patch (pseudo)aneurysm formation. However, risk of heart block is low and long-term outcome is good with the majority of patients reaching Fontan completion. In our opinion, the direct approach appears to be a good and relatively simple procedure in selected cases for the treatment of subaortic stenosis.
对于肺血流无限制且（可能）存在主动脉瓣下狭窄的单心室患者，治疗颇具挑战性，最佳手术策略尚不清楚。由于担心医源性心脏传导阻滞、再梗阻或心室功能障碍，通过扩大室间隔缺损和/或主动脉瓣下腔直接解除主动脉瓣下狭窄的方法，通常已被达穆斯-凯-斯坦塞尔（Damus-Kaye-Stansel）或诺伍德（Norwood）手术所取代。报道直接手术方法长期疗效的研究有限。我们研究的目的是描述和分析我们在单心室患者中直接解除主动脉瓣下狭窄的经验。
收集了1989年至2016年间接受主动脉瓣下狭窄直接解除手术的儿童的人口统计学数据、特征以及术前、术中及术后结果的详细信息。
23例患者（中位年龄：7.4个月，范围：10天至5.5岁）接受了主动脉瓣下狭窄直接解除手术。所有患者均获得完整随访（中位时间：15.6年，范围：34天至26.3年）。7例（30%）患者出现主动脉瓣下狭窄复发。1例（4%）患者发生完全性心脏传导阻滞，1例患者出现中度心室功能障碍。5例（50%）患者在补片和心室切开部位出现（假性）动脉瘤。围手术期有2例死亡。86%的患者成功接受了Fontan手术。
直接解除主动脉瓣下狭窄与再梗阻和补片（假性）动脉瘤形成的重大风险相关。然而，心脏传导阻滞的风险较低，大多数患者Fontan手术完成后的长期疗效良好。我们认为，在治疗主动脉瓣下狭窄的特定病例中，直接手术方法似乎是一种良好且相对简单的手术。
