Magnetic Resonance Unit, National Institute of Cardiology, Warsaw, Poland.
Department of Coronary and Structural Heart Disease, National Institute of Cardiology, Warsaw, Poland.
PLoS One. 2021 Mar 18;16(3):e0248725. doi: 10.1371/journal.pone.0248725. eCollection 2021.
Although hypertrophic cardiomyopathy (HCM) is considered a disease of the left ventricle (LV), right ventricular (RV) abnormalities have also been reported on. Cardiovascular magnetic resonance feature tracking (CMR-FT) accurately and reproducibly quantifies RV myocardial deformation.
To investigate RV deformation disorders in childhood HCM using CMR-FT.
Consecutive subjects aged <18 years with echocardiographic evidence of HCM were enrolled. Cardiovascular magnetic resonance (CMR) was performed including RV volumetric and functional assessment, and late gadolinium enhancement (LGE) imaging.
We included 54 children (37 males, 68.5%) with HCM, of which 28 patients (51.8%; mean extent of 2.18 ± 2.34% of LV mass) had late gadolinium enhancement. LV outflow tract obstruction (LVOTO) was detected in 19 subjects (35.2%). In patients with LVOTO, RV global longitudinal strain (RVGLS) (-16.1±5.0 vs. -20.7±5.3, p<0.01), RVGLS rate (-1.05±0.30 vs. -1.26±0.40, p = 0.03), RV radial strain (RVR) (15.8±7.7 vs. 22.1±7.0, p<0.01) and RVR rate (0.95±0.35 vs. 1.6±0.44, p<0.01) were lower than in patients without LVOTO. The RVR rate (p<0.01) was lower in patients with LGE in comparison to patients without LGE.
Children with HCM, especially with LVOTO, have significantly reduced indices of RV mechanics despite normal RV systolic function. It seems that the degree of LVOT obstruction is responsible for compromising the RV dynamics, rather than either mass or the amount of LV fibrosis.
尽管肥厚型心肌病（HCM）被认为是左心室（LV）的疾病，但也有报道称右心室（RV）存在异常。心血管磁共振特征追踪（CMR-FT）可准确、可重复地定量 RV 心肌变形。
应用 CMR-FT 研究儿童肥厚型心肌病的 RV 变形障碍。
连续纳入经超声心动图证实为 HCM 的年龄<18 岁的患者。进行心血管磁共振（CMR）检查，包括 RV 容积和功能评估以及钆延迟增强（LGE）成像。
我们纳入了 54 例 HCM 患儿（男 37 例，占 68.5%），其中 28 例（51.8%；LV 质量的平均增强程度为 2.18±2.34%）存在 LGE。19 例（35.2%）检测到左心室流出道梗阻（LVOTO）。在 LVOTO 患者中，RV 整体纵向应变（RVGLS）（-16.1±5.0 比-20.7±5.3，p<0.01）、RVGLS 速率（-1.05±0.30 比-1.26±0.40，p=0.03）、RV 径向应变（RVR）（15.8±7.7 比 22.1±7.0，p<0.01）和 RVR 速率（0.95±0.35 比 1.6±0.44，p<0.01）均低于无 LVOTO 的患者。与无 LGE 的患者相比，有 LGE 的患者 RVR 速率（p<0.01）较低。
尽管 RV 收缩功能正常，但患有 HCM 的儿童，尤其是伴有 LVOTO 的患者，RV 力学指标明显降低。似乎 LVOT 梗阻的程度是影响 RV 动力学的原因，而不是 LV 质量或 LV 纤维化的程度。
