Jacquemyn Xander, Long Rita, Rao Sruti, Danford David, Barnes Benjamin T, Kutty Shelby
The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
Pediatr Cardiol. 2025 Jun;46(5):1254-1261. doi: 10.1007/s00246-024-03543-4. Epub 2024 Jun 17.
Echocardiography is pivotal for diagnosis and monitoring of hypertrophic cardiomyopathy (HCM) and can evaluate myocardial function using myocardial work (MW) calculations. Echocardiography is often supplemented by cardiovascular magnetic resonance (CMR) imaging, which can detect myocardial fibrosis using late gadolinium enhancement (LGE). We sought to study the relationship between baseline LGE and MW at baseline and during follow-up in pediatric HCM patients. During the study period (2008-2023), 75 patients were followed up for HCM. In 14 patients (age 14.2 ± 2.8 years, 50.0% male, 6.4 ± 2.9 years follow-up), both LGE-CMR and echocardiography were performed. Global work index (GWI), global constructive work (GCW), global wasted work, and global work efficiency (GWE) were measured, and myocardial fibrosis was estimated by qualitative assessment of LGE. Patients with LGE (n = 7) exhibited significantly impaired baseline MW, including GWI (mean difference, MD - 487.4 mmHg %, 95% CI [- 866.8 mmHg % to - 108.3 mmHg %], p = 0.027), GCW (MD - 536.8 mmHg %, 95% CI [- 929.8 mmHg % to - 144.4 mmHg %], p = 0.020), and GWE (MD - 4.4%, 95% CI [- 8.1% to - 0.7%], p = 0.039). Regional analysis revealed impaired MW indices in segments with LGE, notably basal and mid septal segments. GWI demonstrated high diagnostic performance for LGE presence (sensitivity 93%, specificity 88%, and area under receiver operating characteristic curve 0.85). Baseline LGE presence had no significant impact on MW deterioration during follow-up. MW is significantly impaired in HCM patients with myocardial fibrosis, highlighting potential utility of echocardiography-derived MW analysis as a valuable tool.
超声心动图对于肥厚型心肌病（HCM）的诊断和监测至关重要，并且可以通过心肌做功（MW）计算来评估心肌功能。超声心动图通常辅以心血管磁共振（CMR）成像，后者可利用钆延迟增强（LGE）检测心肌纤维化。我们试图研究儿科HCM患者基线时及随访期间基线LGE与MW之间的关系。在研究期间（2008年至2023年），75例患者因HCM接受随访。在14例患者（年龄14.2±2.8岁，50.0%为男性，随访6.4±2.9年）中，同时进行了LGE-CMR和超声心动图检查。测量了整体做功指数（GWI）、整体建设性做功（GCW）、整体无用功和整体做功效率（GWE），并通过LGE的定性评估来估计心肌纤维化。有LGE的患者（n = 7）表现出基线MW显著受损，包括GWI（平均差值，MD -487.4 mmHg%，95%置信区间[-866.8 mmHg%至-108.3 mmHg%]，p = 0.027）、GCW（MD -536.8 mmHg%，95%置信区间[-929.8 mmHg%至-144.4 mmHg%]，p = 0.020）和GWE（MD -4.4%，95%置信区间[-8.1%至-0.7%]，p = 0.039）。区域分析显示LGE所在节段的MW指标受损，尤其是基底和室间隔中段节段。GWI对LGE存在情况具有较高的诊断效能（敏感性93%，特异性88%，受试者操作特征曲线下面积0.85）。基线LGE的存在对随访期间MW的恶化无显著影响。心肌纤维化的HCM患者MW显著受损，突出了超声心动图衍生的MW分析作为一种有价值工具的潜在效用。
