Barts Heart Centre, London, UK.
Sydney Medical School, University of Sydney, Sydney, Australia.
Eur Heart J Cardiovasc Imaging. 2017 Sep 1;18(9):1034-1040. doi: 10.1093/ehjci/jew187.
Left ventricular papillary muscles (LVPM) can appear disproportionately hypertrophied, particularly in Fabry disease (FD) where storage appears detectable by cardiovascular magnetic resonance (CMR) T1 mapping. The aim of the study was to measure LVPM mass in heart diseases with left ventricular hypertrophy (LVH) and to gain insight into the mechanisms of LVPM hypertrophy in FD.
Four hundred and seventy-eight cases were retrospectively recruited: 125 FD, 85 hypertrophic cardiomyopathy (HCM), 67 amyloid, 82 aortic stenosis (AS), 40 hypertension, 79 controls. LVPM contribution to LVM was manually contoured on CMR short axis cines. T1 values (septal, LVPM) were measured using ShMOLLI sequences in FD and controls. LVPM contribution to LVM was highest in LVH+ve FD and significantly increased compared to all other LVH+ve groups (FD 13 ± 3%, HCM 10 ± 3%, amyloid 8 ± 2%, AS 7 ± 3%, hypertension 7 ± 2%, controls 7 ± 1%; P < 0.001). LVH+ve HCM also had significantly increased LVPM. In LVH-ve cohorts, only FD had significantly increased LVPM (11 ± 3%; P < 0.001). In FD there was concordant septal and LVPM T1. LVH+ve FD: when septal T1 was low, LVPM T1 was low in 90%. LVH-ve FD: when septal T1 was normal, LVPM T1 was normal in 70% (indicating no detectable storage); when septal T1 was low, 75% had low LVPM T1 (indicating storage). LVPM hypertrophy was similar between the low and normal septal T1 groups (11 ± 3% vs. 10 ± 3%, P = 0.08).
Disproportionate hypertrophy of LVPMs in LVH+ve hearts occurred in FD and HCM. This phenomenon also occurred in LVH-ve FD. Low T1 was not always present in FD LVPM hypertrophy, implying additional mechanisms activating hypertrophy signalling pathways.
左心室乳头肌（LVPM）可能会出现不成比例的肥大，特别是在法布里病（FD）中，通过心血管磁共振（CMR）T1 映射可以检测到储存。本研究的目的是测量左心室肥厚（LVH）心脏病患者的 LVPM 质量，并深入了解 FD 中 LVPM 肥大的机制。
回顾性招募了 478 例病例：125 例 FD、85 例肥厚型心肌病（HCM）、67 例淀粉样变性、82 例主动脉瓣狭窄（AS）、40 例高血压、79 例对照。使用 CMR 短轴电影手动描绘 LVPM 对 LVM 的贡献。在 FD 和对照组中使用 ShMOLLI 序列测量 T1 值（间隔、LVPM）。LVPM 对 LVM 的贡献在 LVH+ve FD 中最高，与所有其他 LVH+ve 组相比显著增加（FD 13±3%、HCM 10±3%、淀粉样变性 8±2%、AS 7±3%、高血压 7±2%、对照组 7±1%；P<0.001）。LVH+ve HCM 也有显著增加的 LVPM。在 LVH-ve 队列中，只有 FD 有显著增加的 LVPM（11±3%；P<0.001）。在 FD 中，间隔和 LVPM 的 T1 是一致的。LVH+ve FD：当间隔 T1 较低时，90%的 LVPM T1 也较低。LVH-ve FD：当间隔 T1 正常时，70%的 LVPM T1 正常（表明无明显储存）；当间隔 T1 较低时，75%的 LVPM T1 较低（表明储存）。低和正常间隔 T1 组之间的 LVPM 肥大相似（11±3%比 10±3%，P=0.08）。
LVH+ve 心脏中 LVPM 的不成比例肥大发生在 FD 和 HCM 中。这种现象也发生在 LVH-ve FD 中。FD 的 LVPM 肥大并不总是存在低 T1，这意味着存在激活肥大信号通路的其他机制。
