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Outcomes in Patients With Hypertrophic Cardiomyopathy and Left Ventricular Systolic Dysfunction
Journal of the American College of Cardiology ( IF 21.7 ) Pub Date : 2020-06-01 , DOI: 10.1016/j.jacc.2020.04.045
Ethan J Rowin 1 , Barry J Maron 1 , Richard T Carrick 1 , Parth P Patel 1 , Ben Koethe 2 , Sophie Wells 1 , Martin S Maron 1
Affiliation  

BACKGROUND End-stage (ES) hypertrophic cardiomyopathy (HCM) has been considered a particularly grim and unfavorable disease complication, associated with substantial morbidity and mortality, frequently requiring heart transplant. Previous reports have included small numbers of patients with relatively short follow-up, predominantly in prior treatment eras. OBJECTIVES The purpose of this study was to re-evaluate clinical profile and prognosis for end-stage heart failure in a large HCM cohort with contemporary management strategies. METHODS Patients at Tufts HCM Institute, from 2004 to 2017, were identified with ES and systolic dysfunction (ejection fraction [EF] <50%), followed for 5.8 ± 4.7 years (up to 18 years). RESULTS Of the 2,447 patients, 118 (4.8%) had ES-HCM (EF 39 ± 9%; range 12% to 49%) at age 48 ± 15 years. Notably, over follow-up, 57 patients (48%) achieved clinical stability in New York Heart Association functional classes I/II with medical treatment (or cardiac resynchronization therapy), including 6 patients ≥10 years from ES diagnosis (up to 14 years). In total, 61 other patients (52%) developed refractory heart failure to disabling New York Heart Association functional classes III/IV (5.2%/year); 67% have survived, including 31 with heart transplant. Of the 118 ES patients, 21 had appropriate implantable cardioverter-defibrillator (ICD) therapy terminating potentially lethal tachyarrhythmias, with no difference in frequency of events in patients with EF 35% to 49% versus EF <35% (17% vs. 19%; p = 0.80). With all available treatment modalities, ES-related mortality was 1.9%/year, with 10-year survival of 85% (95% confidence interval: 77% to 94%). Mortality was 4-fold lower than previously reported for ES (8.0%/year), but exceeded 10-fold HCM with preserved EF (0.2%/year; p < 0.001). CONCLUSIONS Although ES remains an important complication of HCM, contemporary treatment strategies, including ICDs and heart transplant, are associated with significantly lower mortality than previously considered. Primary prevention ICDs should be considered when EF is <50% in HCM. Rapid heart failure progression is not an inevitable consequence of ES, and some patients experience extended periods of clinical stability.

中文翻译:

肥厚型心肌病和左心室收缩功能障碍患者的预后

背景终末期(ES)肥厚型心肌病(HCM)被认为是一种特别严峻和不利的疾病并发症,与大量的发病率和死亡率相关,经常需要心脏移植。以前的报告包括少数患者,随访时间相对较短,主要是在以前的治疗时代。目的 本研究的目的是通过现代管理策略重新评估大型 HCM 队列中终末期心力衰竭的临床特征和预后。方法 2004 年至 2017 年,塔夫茨 HCM 研究所的患者被确定为 ES 和收缩功能障碍(射血分数 [EF] <50%),随访时间为 5.8 ± 4.7 年(最长 18 年)。结果 在 2,447 名患者中,118 名 (4.8%) 在 48 ± 15 岁时患有 ES-HCM(EF 39 ± 9%;范围为 12% 至 49%)。值得注意的是,在后续跟踪中,57 名患者(48%)通过药物治疗(或心脏再同步治疗)达到纽约心脏协会功能分级 I/II 的临床稳定性,其中 6 名患者从 ES 诊断起≥10 年(最长 14 年)。总共有 61 名其他患者 (52%) 发生了顽固性心力衰竭,导致纽约心脏协会功能分级为 III/IV 级(5.2%/年);67% 的人幸存下来,其中 31 人接受了心脏移植手术。在 118 名 ES 患者中,21 名接受了适当的植入式心律转复除颤器 (ICD) 治疗以终止可能致命的快速性心律失常,EF 35% 至 49% 与 EF <35% 患者的事件频率没有差异(17% 与 19% ; p = 0.80)。在所有可用的治疗方式下,ES 相关死亡率为 1.9%/年,10 年生存率为 85%(95% 置信区间:77% 至 94%)。死亡率比先前报告的 ES 低 4 倍(8.0%/年),但超过 10 倍的 HCM 保留 EF(0.2%/年;p < 0.001)。结论 尽管 ES 仍然是 HCM 的一个重要并发症,但现代治疗策略,包括 ICD 和心脏移植,与之前考虑的死亡率显着降低相关。当 HCM 中 EF <50% 时,应考虑一级预防 ICD。快速心力衰竭进展不是 ES 的必然结果,一些患者经历了长时间的临床稳定。与比以前认为的显着降低的死亡率相关。当 HCM 中 EF <50% 时,应考虑一级预防 ICD。快速心力衰竭进展不是 ES 的必然结果,一些患者经历了长时间的临床稳定。与比以前认为的显着降低的死亡率相关。当 HCM 中 EF <50% 时,应考虑一级预防 ICD。快速心力衰竭进展不是 ES 的必然结果,一些患者经历了长时间的临床稳定。
更新日期:2020-06-01
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