From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
Circ Arrhythm Electrophysiol. 2016 Jun;9(6). doi: 10.1161/CIRCEP.115.003818.
Hypertrophic cardiomyopathy is associated with sudden cardiac death (SCD). Some studies have shown an association between risk of sudden death and left ventricular maximal wall thickness (MWT), but there are few data in patients with extreme hypertrophy. The aim of this study was to determine the relation between MWT and the risk of SCD.
This is a multicenter, retrospective, longitudinal cohort study of 3673 adult (≥16 years) patients, previously used to develop and validate a risk prediction model for SCD (HCM Risk-SCD [hypertrophic cardiomyopathy risk-SCD]). There was an inverted U-shaped relation between MWT and the estimated 5-year risk of SCD. In patients with MWT≥35 mm (n=47; mean age, 33 years; 81% men), there was a single SCD end point (annual rate, 0.2%; 95% confidence interval, 0.03-1.60) and 3 additional cardiovascular events during a median follow-up of 9.5 years. Compared with patients with MWT≤14 mm, those with MWT≥35 mm did not have a higher risk for SCD (hazard ratio, 0.22; 95% confidence interval, 0.03-1.65), cardiovascular death (hazard ratio, 0.66; 95% confidence interval, 0.26-1.67), or all-cause mortality (hazard ratio, 0.73; 95% confidence interval, 0.32-1.69).
The risk of SCD has a complex, nonlinear relationship to MWT. The pathophysiological mechanisms behind this observation require further study but implantable cardioverter defibrillator implantation should not be guided solely on the severity of left ventricular hypertrophy.
肥厚型心肌病与心脏性猝死（SCD）有关。一些研究表明，左心室最大壁厚度（MWT）与猝死风险之间存在关联，但在极度肥厚的患者中数据较少。本研究旨在确定 MWT 与 SCD 风险之间的关系。
这是一项多中心、回顾性、纵向队列研究，共纳入 3673 名成年（≥16 岁）患者，此前曾用于开发和验证 SCD 风险预测模型（HCM Risk-SCD [肥厚型心肌病风险-SCD]）。MWT 与估计的 5 年 SCD 风险之间呈倒 U 形关系。在 MWT≥35mm（n=47；平均年龄 33 岁；81%为男性）的患者中，发生单一 SCD 终点事件（年发生率为 0.2%；95%置信区间，0.03-1.60），在中位数为 9.5 年的随访期间，还发生了 3 例额外的心血管事件。与 MWT≤14mm 的患者相比，MWT≥35mm 的患者 SCD（风险比，0.22；95%置信区间，0.03-1.65）、心血管死亡（风险比，0.66；95%置信区间，0.26-1.67）或全因死亡率（风险比，0.73；95%置信区间，0.32-1.69）的风险均无显著升高。
SCD 的风险与 MWT 呈复杂的非线性关系。这种观察结果背后的病理生理机制需要进一步研究，但植入式心脏复律除颤器的植入不应仅基于左心室肥厚的严重程度来指导。
