Pediatric Cardiology Department, Sant Joan de Déu Children Hospital, C/Passeig Sant Joan de Deu, s/n, 08950, Esplugues, Barcelona, Spain.
CMR Imaging Department, Sant Joan de Déu Children Hospital, Barcelona, Spain.
Pediatr Cardiol. 2021 Aug;42(6):1324-1333. doi: 10.1007/s00246-021-02615-z. Epub 2021 May 2.
Right ventricular (RV) dilatation is the determining prognostic factor in the long-term follow up of patients with repaired Tetralogy of Fallot (TOF). The objective of this study is to analyze whether the results vary depending on the timing of the complete repair and on the surgical technique applied.
MATERIAL-METHODS: This is a retrospective longitudinal study in which patients with standard TOF were divided into 3 groups depending on their age at surgical repair: group 1 = Early repair (n = 12,1-8 months), group 2 = Late repair (n = 26, > 8 months), and group 3 = Late repair with previous palliative surgery (n = 17, > 8 months). Clinical, echocardiographic and cardiac magnetic resonance (CMR) data from patients that had received complete reparative surgery in our institution from January 2000 to March 2014 were analyzed and compared.
55 patients with echocardiogram and CMR studies (13.39 ± 3.59 years) were reviewed. All patients had at least moderate pulmonary regurgitation (PR). We observed a positive correlation between PR and right ventricular end-diastolic volume (r = 0.418; p = 0,004). Group 3 had more severe right ventricular dilatation than patients in groups 1 and 2 (p = 0.001). No differences in right ventricular end-diastolic volume, PR, and pulmonary trunk dimensions were observed between groups 1 and 2. Patients in group 3 had a longer hospital stay.
Although all patients from our cohort had significant PR, age at surgery was not related to RV or pulmonary trunk dilatation. Previous palliative surgery was associated with more severe right ventricular dilatation and longer hospital stays. No differences were observed between early and late repair groups. Our study suggests that postponing TOF repair to a late stage does not improve the degree of PR or long-term morbidity from RV dilatation. Palliative surgery should be avoided if possible.
右心室(RV)扩张是法洛四联症(TOF)患者长期随访中决定预后的因素。本研究的目的是分析手术修复的时机和所应用的手术技术是否会影响结果。
这是一项回顾性纵向研究,将具有标准 TOF 的患者根据手术修复时的年龄分为 3 组:组 1=早期修复(n=12,1-8 个月),组 2=晚期修复(n=26,>8 个月),组 3=晚期修复伴先前姑息性手术(n=17,>8 个月)。分析比较了 2000 年 1 月至 2014 年 3 月期间在我们医院接受过完全修复手术的患者的临床、超声心动图和心脏磁共振(CMR)数据。
共回顾了 55 例接受超声心动图和 CMR 检查的患者(13.39±3.59 岁)。所有患者均存在至少中度的肺动脉瓣反流(PR)。我们观察到 PR 与右心室舒张末期容积(RVEDV)呈正相关(r=0.418;p=0.004)。与组 1 和组 2 相比,组 3 的右心室扩张更为严重(p=0.001)。组 1 和组 2 之间的 RVEDV、PR 和肺动脉干直径无差异。组 3 的患者住院时间较长。
尽管我们队列中的所有患者均存在显著的 PR,但手术时的年龄与 RV 或肺动脉干扩张无关。先前的姑息性手术与更严重的右心室扩张和更长的住院时间有关。早期修复组和晚期修复组之间无差异。我们的研究表明,将 TOF 修复推迟到晚期并不会改善 PR 程度或 RV 扩张的长期发病率。如果可能的话,应避免姑息性手术。