Alsaied Tarek, Castrillon Carlos Diaz, Christopher Adam, Da Silva Jose, Morell Victor O, Lanford Lizabeth, Goldstein Bryan H, Feingold Brian, Seery Thomas, Arora Gaurav, Hoskoppal Arvind, Johnson Jennifer A, Tadros Sameh, Olivieri Laura J, Da Silva Luciana De Fonseca
Heart Institute, UPMC Children's Hospital of Pittsburgh, Division of Pediatric Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
DaSilva Center of Ebstein Anomaly, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Int J Cardiol Congenit Heart Dis. 2022 Jan 31;7:100342. doi: 10.1016/j.ijcchd.2022.100342. eCollection 2022 Mar.
Despite the clinical benefits of the cone operation for Ebstein's anomaly, significant right ventricular (RV) dysfunction is frequently seen immediately after the procedure and if persistent may portend worse long-term outcomes. In this study we sought to evaluate the predictors of RV dysfunction after the cone operation using preoperative CMR.
This was a retrospective review of 26 consecutive patients who had the cone operation. Patients with significant RV dysfunction (RVD), defined as moderate or severe dysfunction by discharge echocardiogram, were compared to patients with no or mild dysfunction (no RVD).
The median age at the operation was 12.2 years (interquartile range (IQR): 4.9-31.7 years). Eighteen patients (69%) had RVD. Patients with RVD had worse preoperative RV ejection fraction (36 ± 15 vs 49 ± 11%, p = 0.02) and a larger cardiothoracic (CT) index (44 ± 8 vs 37 ± 6, p = 0.03). The tricuspid valve was more severely abnormal in the RVD group with higher rotational angle (45 ± 17 vs 23 ± 10°, 0.03) and higher displacement index (39 ± 18 vs 23 ± 12%, p = 0.02). RVD associated with a higher vasoactive inotropic score (P < 0.01) and a trend towards a longer intensive care stay (p = 0.07).
RVD is common after the cone operation and associated with higher need for postoperative inotropes. Predictors include lower preoperative RV ejection fraction, a more dilated heart and more severe tricuspid valve abnormality. Preoperative CMR is an important tool in preoperative assessment and helps predict RVD.
尽管圆锥手术治疗埃布斯坦畸形有临床益处，但术后常立即出现明显的右心室（RV）功能障碍，若持续存在可能预示更差的长期预后。在本研究中，我们试图利用术前心脏磁共振成像（CMR）评估圆锥手术后RV功能障碍的预测因素。
这是一项对26例连续接受圆锥手术患者的回顾性研究。将出院时经超声心动图定义为中度或重度功能障碍的显著RV功能障碍（RVD）患者与无或轻度功能障碍（无RVD）患者进行比较。
手术时的中位年龄为12.2岁（四分位间距（IQR）：4.9 - 31.7岁）。18例患者（69%）有RVD。RVD患者术前RV射血分数更低（36 ± 15 vs 49 ± 11%，p = 0.02），心胸（CT）指数更大（44 ± 8 vs 37 ± 6，p = 0.03）。RVD组三尖瓣异常更严重，旋转角度更高（45 ± 17 vs 23 ± 10°，p = 0.03），移位指数更高（39 ± 18 vs 23 ± 12%，p = 0.02）。RVD与更高的血管活性正性肌力评分相关（P < 0.01），且有重症监护停留时间更长的趋势（p = 0.07）。
圆锥手术后RVD很常见，且与术后更高的正性肌力药物需求相关。预测因素包括术前较低的RV射血分数、心脏更扩张和更严重的三尖瓣异常。术前CMR是术前评估的重要工具，有助于预测RVD。
