Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
Department of Paediatric Cardiology, Evelina London Children's Hospital, London, UK.
Heart. 2018 Sep;104(17):1411-1416. doi: 10.1136/heartjnl-2017-312195. Epub 2017 Dec 28.
Infants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation.
This multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life.
Median age at definitive surgery was 179 (range 0-357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1-11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation.
Although surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.
患有症状性部分和过渡性房室间隔缺损并接受早期手术修复的婴儿被认为风险更大。然而，该队列患者的预后和风险特征定义不明确。本研究旨在调查接受早期修复的有症状婴儿的结果，并确定可能预测死亡率和再次手术的风险因素。
这项多中心研究在 2000 年至 2015 年间在三个三级中心招募了 51 名患者（24 名女性）。纳入标准如下：（1）部分和过渡性房室间隔缺损，（2）对治疗无反应的心力衰竭，（3）在生命的第一年进行双心室修复。
明确手术的中位年龄为 179 天（范围 0-357 天）。16 名患者（31%）的左房室瓣解剖结构不良：发育不良（n=7）、双孔（n=3）、瓣叶严重缺陷（n=1）、左房室瓣孔和/或壁瓣发育不良（n=3）、短/定义不清的腱索（n=2）。初次修复后有 3 例院内死亡（5.9%）。11 名患者（22%）因严重左房室瓣反流和/或狭窄，在中位间隔 40 天（4 天至 5.1 年）再次手术。1 名患者需要机械替代左房室瓣。中位随访 3.8 年后（0.1-11.4 年），所有患者均为纽约心脏协会（NYHA）I 级。多变量分析显示，左房室瓣解剖结构不良是与左房室瓣再次手术相关的唯一危险因素。
尽管大多数情况下手术修复成功，但在婴儿期接受手术修复的部分和过渡性房室间隔缺损患者经历了显著的发病率和死亡率。具有不良左房室瓣解剖结构的患者再次手术率较高。
