Kim Hannah S, Abarbanell Ginnie, Simpson Kathleen, Abarbanell Aaron M, Eghtesady Pirooz, Levy Philip T, Singh Gautam K
Department of Pediatrics, Hackensack Meridian School of Medicine, HMH Joseph M. Sanzari Children's Hospital, Hackensack, NJ 07601, USA.
Department of Pediatrics, UT Health, San Antonio, TX 78229, USA.
J Clin Med. 2025 Jun 29;14(13):4602. doi: 10.3390/jcm14134602.
Patients with single right ventricular morphology (SRV) may exhibit impaired function with increased morbidity, mortality, and need for cardiac transplant due to progressive SRV failure after the Fontan procedure. The aim of the study was to longitudinally characterize the cardiac mechanics and trajectory of disease evolution of SRV failure in Fontan patients. : We performed a case-controlled longitudinal study of 52 patients who underwent extracardiac Fontan palliation for SRV between 1994 and 2015 and compared echocardiographic measures of right ventricular (RV) function, RV-systemic vascular coupling and ventricular remodeling between patients who required heart transplants due to SRV failure (study group, = 26) and those who did not (control group, = 26). To define the trajectory, measurements were obtained at four matching time points equivalent in duration from Fontan. : RV circumferential shortening function declined in both groups over the time period, but was significantly lower ( < 0.01) in the study group farther from the Fontan. RV-systemic vascular coupling, assessed by systolic time interval measures and RV work, was preserved in the control group, but significantly altered ( < 0.001) in the study group. Relative wall thickness decreased, and the minor/major-axis ratio, as an index of ventricular geometry, increased in the study group, but both remained stable in the control group. : This study suggests that positive ventricular remodeling with enhanced circumferential systolic function, and preserved RV-vascular coupling, appear to be adaptive and protective mechanisms against RV failure in Fontan with SRV. These indices of cardiac mechanics may serve as clinically relevant quantifiable markers of disease evolution, and early indicators for therapeutic intervention.
具有单一右心室形态（SRV）的患者可能会出现功能受损，由于Fontan手术后SRV进行性衰竭，发病率、死亡率增加，且需要进行心脏移植。本研究的目的是纵向描述Fontan患者SRV衰竭的心脏力学和疾病演变轨迹。我们对1994年至2015年间因SRV接受心外Fontan姑息手术的52例患者进行了病例对照纵向研究，比较了因SRV衰竭需要心脏移植的患者（研究组，n = 26）和未需要心脏移植的患者（对照组，n = 26）之间右心室（RV）功能、RV-体循环血管耦合和心室重构的超声心动图测量结果。为了确定轨迹，在与Fontan手术持续时间相等的四个匹配时间点进行测量。在该时间段内，两组的RV圆周缩短功能均下降，但研究组中距离Fontan手术时间更远时，该功能显著更低（P < 0.01）。通过收缩期时间间隔测量和RV作功评估的RV-体循环血管耦合在对照组中得以保留，但在研究组中显著改变（P < 0.001）。研究组的相对壁厚度降低，作为心室几何形状指标的短轴/长轴比值增加，但在对照组中两者均保持稳定。本研究表明，具有增强的圆周收缩功能和保留的RV-血管耦合的正向心室重构似乎是Fontan合并SRV患者对抗RV衰竭的适应性和保护机制。这些心脏力学指标可作为疾病演变的临床相关可量化标志物以及治疗干预的早期指标。
