NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK.
Department of CMR, Royal Brompton Hospital, Sydney Street, Sydney, SW3 6NP, UK.
J Cardiovasc Magn Reson. 2021 Mar 8;23(1):26. doi: 10.1186/s12968-021-00720-9.
Heart failure (HF) in hypertrophic cardiomyopathy (HCM) is associated with high morbidity and mortality. Predictors of HF, in particular the role of myocardial fibrosis and microvascular ischemia remain unclear. We assessed the predictive value of cardiovascular magnetic resonance (CMR) for development of HF in HCM in an observational cohort study.
Serial patients with HCM underwent CMR, including adenosine first-pass perfusion, left atrial (LA) and left ventricular (LV) volumes indexed to body surface area (i) and late gadolinium enhancement (%LGE- as a % of total myocardial mass). We used a composite endpoint of HF death, cardiac transplantation, and progression to NYHA class III/IV.
A total of 543 patients with HCM underwent CMR, of whom 94 met the composite endpoint at baseline. The remaining 449 patients were followed for a median of 5.6 years. Thirty nine patients (8.7%) reached the composite endpoint of HF death (n = 7), cardiac transplantation (n = 2) and progression to NYHA class III/IV (n = 20). The annual incidence of HF was 2.0 per 100 person-years, 95% CI (1.6-2.6). Age, previous non-sustained ventricular tachycardia, LV end-systolic volume indexed to body surface area (LVESVI), LA volume index ; LV ejection fraction, %LGE and presence of mitral regurgitation were significant univariable predictors of HF, with LVESVI (Hazard ratio (HR) 1.44, 95% confidence interval (95% CI) 1.16-1.78, p = 0.001), %LGE per 10% (HR 1.44, 95%CI 1.14-1.82, p = 0.002) age (HR 1.37, 95% CI 1.06-1.77, p = 0.02) and mitral regurgitation (HR 2.6, p = 0.02) remaining independently predictive on multivariable analysis. The presence or extent of inducible perfusion defect assessed using a visual score did not predict outcome (p = 0.16, p = 0.27 respectively).
The annual incidence of HF in a contemporary ambulatory HCM population undergoing CMR is low. Myocardial fibrosis and LVESVI are strongly predictive of future HF, however CMR visual assessment of myocardial perfusion was not.
肥厚型心肌病（HCM）患者并发心力衰竭（HF）与较高的发病率和死亡率相关。HF 的预测因素，尤其是心肌纤维化和微血管缺血的作用仍不明确。我们在一项观察性队列研究中评估了心血管磁共振（CMR）对 HCM 患者 HF 发生的预测价值。
连续纳入 HCM 患者行 CMR 检查，包括腺苷首过灌注、左心房（LA）和左心室（LV）容积指数化（i）和晚期钆增强（%LGE-作为总心肌质量的百分比）。我们使用 HF 死亡、心脏移植和进展至纽约心脏协会（NYHA）心功能分级 III/IV 的复合终点作为评估指标。
共 543 例 HCM 患者接受了 CMR 检查，其中 94 例在基线时达到了复合终点。其余 449 例患者的中位随访时间为 5.6 年。39 例患者（8.7%）达到 HF 死亡（n=7）、心脏移植（n=2）和 NYHA 心功能分级 III/IV 进展（n=20）的复合终点。HF 的年发生率为 2.0/100 人年，95%可信区间（95%CI）为 1.6-2.6。年龄、既往非持续性室性心动过速、LV 末收缩容积指数化（LVESVI）、LA 容积指数、LV 射血分数、%LGE 和二尖瓣反流是 HF 的显著单变量预测因素，LVESVI（危险比（HR）1.44，95%置信区间（95%CI）1.16-1.78，p=0.001）、%LGE 每增加 10%（HR 1.44，95%CI 1.14-1.82，p=0.002）、年龄（HR 1.37，95%CI 1.06-1.77，p=0.02）和二尖瓣反流（HR 2.6，p=0.02）在多变量分析中仍独立预测 HF。使用视觉评分评估的诱发性灌注缺陷的存在或程度与结局无关（p=0.16，p=0.27）。
在接受 CMR 的当代门诊 HCM 人群中，HF 的年发生率较低。心肌纤维化和 LVESVI 是 HF 的强烈预测因素，但 CMR 视觉评估心肌灌注无预测价值。
