Division of Cardiology, The Children's Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, 34th and Civic Center Boulevard, Philadelphia, PA, 19104, USA.
Tufts University School of Medicine, Boston, MA, 02116, USA.
Int J Cardiovasc Imaging. 2020 Sep;36(9):1667-1677. doi: 10.1007/s10554-020-01870-0. Epub 2020 May 3.
The principal aim of this study was to evaluate changes in systolic function in the single right ventricle (SRV), during progression of the same patient through the three stages of surgical repair for hypoplastic left heart syndrome and during a 5-year follow-up. We hypothesize that, SRV global longitudinal strain (GLS) will be low during 3 stages of repair even in stable patients. We retrospectively evaluated 140 echocardiograms in 20 patients with HLHS (ages 0-11.3 years), before and after 3 stages of surgical palliation. Five-year follow-up data were available in all 20 patients. Controls with structurally normal hearts and in the same age group were used for comparison. We utilized speckle-tracking imaging for assessment of SRV segmental and global longitudinal and circumferential strains, from previously acquired 4-chamber and mid-cavity short-axis views prior to and within 1-3 months of each surgical stage. Longitudinal strain (LS) remained low through all 3 stages of repair and during follow-up. The pre-Fontan stage demonstrated significant interstage improvement compared to the post-Glenn stage despite similar volume status. Global LS was (- 15.6 ± 4.5% after Fontan surgery and remained similar (- 15.32 ± 3.2%) 5 years later. The SRV also showed increased dominance of circumferential strain compared to the normal RV, where the longitudinal deformation was dominant. In SRV, longitudinal strain may be a useful clinical index for evaluating both segmental and global function in an objective manner. Due to lack of significant clinical deterioration over a 10-year period, we speculate that a "lower-than-normal" longitudinal strain may be used as an objective measure of SRV function in clinically stable patients, particularly after the Fontan operation. Compensatory mechanisms where the longitudinal pattern of contraction switches to a more circumferential pattern, may play a role in asymptomatic patients with HLHS.
本研究的主要目的是评估在同种患者经历左心发育不全综合征（HLHS）的三种手术修复阶段以及 5 年随访期间，单右心室（SRV）收缩功能的变化。我们假设，即使在稳定的患者中，SRV 整体纵向应变（GLS）在修复的三个阶段也会较低。我们回顾性评估了 20 例 HLHS 患者（年龄 0-11.3 岁）的 140 次超声心动图，这些患者在经历了 3 个阶段的手术姑息治疗前后。所有 20 例患者均获得了 5 年的随访数据。我们使用斑点追踪成像来评估 SRV 节段和整体纵向及圆周应变，从之前获得的 4 腔心和中腔短轴视图中获取，这些视图是在每次手术阶段之前和 1-3 个月内获得的。纵向应变（LS）在所有三个修复阶段以及随访期间均保持较低水平。尽管容量状态相似，但在 Fontan 手术前阶段与 Glenn 手术后阶段相比，各个阶段之间仍有显著的改善。Fontan 手术后的整体 LS 为（-15.6 ± 4.5%），5 年后仍保持相似（-15.32 ± 3.2%）。SRV 也显示出与正常 RV 相比，圆周应变的优势增加，其中纵向变形为主导。在 SRV 中，纵向应变可能是一种有用的临床指标，可以客观地评估节段和整体功能。由于在 10 年期间没有明显的临床恶化，我们推测在临床上稳定的患者，特别是在 Fontan 手术后，“低于正常”的纵向应变可作为 SRV 功能的客观测量指标。在无症状的 HLHS 患者中，纵向收缩模式向更圆周收缩模式转换的代偿机制可能起作用。
