Cardiology, CHU Toulouse Pôle Cardiovasculaire et Métabolique, Toulouse, France
Cardiac Imaging Centre, CHU Toulouse Pôle Cardiovasculaire et Métabolique, Toulouse, France.
Open Heart. 2021 Jan;8(1). doi: 10.1136/openhrt-2020-001462.
To phenotype patients referred to a tertiary centre for the exploration of a left ventricular hypertrophy (LVH) starting from 12 mm of left ventricular wall thickness (LVWT).
Consecutive patients referred for aetiological workup of LVH, beginning at 12 mm of LVWT were retrospectively included in this tertiary single-centred observational study. Patients presenting with severe aortic stenosis were excluded. Aetiological workup was reviewed for each subject and aetiologies were adjudicated by expert consensus.Among 591 patients referred for LVH aetiological workup, 41% had a maximal LVWT below 15 mm. LVH aetiologies were led by cardiac amyloidosis (CA, 34.3%), followed by sarcomeric hypertrophic cardiomyopathy (S-HCM, 32.1%), hypertensive cardiomyopathy (21.7%), unknown aetiology (7.6%) and other (4.2%), including Anderson-Fabry's disease (1.7%). CA and S-HCM affected over 50% of patients with mild LVH (12-14 mm); the prevalence of these aetiologies rose with LVH severity. Among patients with Anderson-Fabry's disease, 4 (40%) had a maximal LVWT <15 mm.
Mild LVH (ie, 12-14 mm) conceals multiple aetiologies that can lead to specific treatment, cascade family screening and specific follow-up. Overall, CA is nowadays the leading cause of LVH in tertiary centers.
从左心室壁厚度（LVWT）12 mm 开始，对转诊至三级中心进行左心室肥厚（LVH）探索的患者进行表型分析。
本项回顾性三级单中心观察性研究纳入了连续转诊至三级中心进行 LVH 病因学检查的患者，LVWT 起始值为 12 mm。排除了严重主动脉瓣狭窄的患者。对每位患者的病因学检查进行回顾，并通过专家共识对病因进行裁决。在 591 例转诊至三级中心进行 LVH 病因学检查的患者中，41%的患者最大 LVWT 低于 15 mm。LVH 的病因主要为心脏淀粉样变性（CA，34.3%），其次为肌节性肥厚型心肌病（S-HCM，32.1%）、高血压性心肌病（21.7%）、不明原因（7.6%）和其他（4.2%），包括安德森-法布里病（1.7%）。CA 和 S-HCM 影响超过 50%的轻度 LVH（12-14 mm）患者；这些病因的患病率随着 LVH 严重程度的增加而上升。在安德森-法布里病患者中，4 例（40%）最大 LVWT <15 mm。
轻度 LVH（即 12-14 mm）隐匿多种病因，可进行针对性治疗、级联家族筛查和特定随访。总的来说，CA 是目前三级中心 LVH 的主要病因。
