Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA.
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA; Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, MN, USA; Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Labotorary, Mayo Clinic, Rochester, MN, USA.
Mayo Clin Proc. 2022 Jun;97(6):1123-1133. doi: 10.1016/j.mayocp.2021.12.005. Epub 2022 Apr 27.
To assess whether right ventricular enlargement (RVE) and right ventricular dysfunction (RVD) adversely affect prognosis in hypertrophic cardiomyopathy (HCM).
Data were retrieved from Mayo Clinic's prospectively collected HCM registry between January 1, 2000, and September 30, 2012. Right ventricle (RV) size and function were semiquantitatively categorized via echocardiography as normal (RV-Norm) versus abnormal (RV-Abn) (RVE or RVD). All-cause mortality was the primary endpoint.
Of 1878 HCM patients studied (mean age 53±15 years; 41.6% female), only 71 (3.8%) had RV-Abn (24 RVE, 28 RVD, 19 combined RVE and RVD). Compared with HCM patients with RV-Norm, RV-Abn patients were older (57±14 vs 53±15 years, P=.02), more symptomatic (New York Heart Association functional class III-IV in 62.0% vs 48.6%, P=.03), had more atrial fibrillation (53.5% vs 17.3%, P<.001), and more prior implantable cardioverter-defibrillator implantation (23.9% vs 11.3%, P=.02). Median follow-up was 9.4 years with 311 deaths. Patients who were RV-Abn had higher all-cause mortality compared with RV-Norm (log-rank P<.001); 24.1% (95% CI, 15.5% to 35.3%) vs 6.1% (95% CI, 5.1% to 7.3%) at 5 years. In multivariable Cox modeling, RV-Abn (hazard ratio, 1.89; 95% CI, 1.18 to 3.03; P=.008) was associated independently with all-cause mortality after adjusting for age, female sex, New York Heart Association functional class, atrial fibrillation, hypertension, coronary artery disease, implantable cardioverter-defibrillator implantation, beta blocker use, prior septal reduction therapy, resting LV outflow tract gradient, maximal LV wall thickness, and moderate or greater tricuspid regurgitation.
Although perturbations in RV size and function were observed in fewer than 5% of patients with HCM, they were associated with nearly two-fold higher all-cause mortality at long-term follow-up.
评估右心室扩大（RVE）和右心室功能障碍（RVD）是否对肥厚型心肌病（HCM）的预后产生不利影响。
本研究数据来自梅奥诊所前瞻性收集的 HCM 注册中心，时间为 2000 年 1 月 1 日至 2012 年 9 月 30 日。通过超声心动图将右心室（RV）大小和功能半定量分类为正常（RV-Norm）与异常（RV-Abn）（RVE 或 RVD）。全因死亡率是主要终点。
在研究的 1878 例 HCM 患者中（平均年龄 53±15 岁；41.6%为女性），只有 71 例（3.8%）存在 RV-Abn（24 例 RVE、28 例 RVD、19 例 RVE 和 RVD 合并）。与 RV-Norm 的 HCM 患者相比，RV-Abn 患者年龄更大（57±14 岁 vs 53±15 岁，P=.02），症状更明显（纽约心脏协会功能分级 III-IV 级的患者占 62.0% vs 48.6%，P=.03），心房颤动发生率更高（53.5% vs 17.3%，P<.001），植入式心脏复律除颤器植入术的比例更高（23.9% vs 11.3%，P=.02）。中位随访时间为 9.4 年，有 311 例死亡。与 RV-Norm 相比，RV-Abn 患者的全因死亡率更高（对数秩检验 P<.001）；5 年时分别为 24.1%（95%CI，15.5%至 35.3%）和 6.1%（95%CI，5.1%至 7.3%）。多变量 Cox 模型分析显示，在校正年龄、女性、纽约心脏协会功能分级、心房颤动、高血压、冠状动脉疾病、植入式心脏复律除颤器植入、β受体阻滞剂使用、既往间隔部肥厚减容治疗、静息左室流出道梯度、最大左室壁厚度和中重度三尖瓣反流后，RV-Abn（风险比，1.89；95%CI，1.18 至 3.03；P=.008）与全因死亡率独立相关。
尽管 HCM 患者中不到 5%存在 RV 大小和功能的改变，但在长期随访中，这些改变与近两倍的全因死亡率相关。
