Hebl Virginia B, Miranda William R, Ong Kevin C, Hodge David O, Bos J Martijn, Gentile Federico, Klarich Kyle W, Nishimura Rick A, Ackerman Michael J, Gersh Bernard J, Ommen Steve R, Geske Jeffrey B
Department of Medicine/Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN.
Department of Health Sciences Research, Mayo Clinic, Jacksonville, FL.
Mayo Clin Proc. 2016 Mar;91(3):279-87. doi: 10.1016/j.mayocp.2016.01.002.
To describe the survival of a large nonobstructive hypertrophic cardiomyopathy (NO-HCM) cohort and to identify risk factors for increased mortality in this population.
Patients were identified from the Mayo Clinic HCM database from January 1, 1975, through November 30, 2006, for this retrospective observational study. Patients with resting or provocable left ventricular outflow tract gradients were excluded. Echocardiographic, clinical, and genetic data were compared between subgroups, and survival data were compared with expected population rates.
A total of 706 patients with NO-HCM were identified. During median follow-up of 5 years (mean, 7 years), there were 208 deaths. Overall survival was no different than expected compared with age- and sex-matched white US population mortality rates (P=.77). Independent predictors of death were age at diagnosis, "burned out" HCM, and history of transient ischemic attack or stroke; use of an implantable cardioverter defibrillator (ICD) was inversely related to death. After exclusion of patients with an ICD, there was no difference in survival compared with age- and sex- matched individuals (P=.39); age, previous transient ischemic attack/stroke, and burned out HCM were predictors of death.
In this cohort, patients with NO-HCM had similar survival rates as age- and sex-matched white US population mortality rates. Although use of an ICD was inversely related to death, no differences in overall survival were seen after those patients were excluded. Burned out HCM was independently associated with an increased risk of death, identifying a subset of patients who may benefit from more aggressive therapies.
描述一大群非梗阻性肥厚型心肌病（NO-HCM）患者的生存情况，并确定该人群死亡率增加的危险因素。
通过对梅奥诊所HCM数据库在1975年1月1日至2006年11月30日期间的数据进行回顾性观察研究来确定患者。排除静息或激发状态下左心室流出道有压力阶差的患者。对亚组间的超声心动图、临床和基因数据进行比较，并将生存数据与预期的人群率进行比较。
共确定了706例NO-HCM患者。在中位随访5年（平均7年）期间，有208例死亡。与年龄和性别匹配的美国白人人群死亡率相比，总体生存率无差异（P = 0.77）。死亡的独立预测因素为诊断时的年龄、“终末期”HCM、短暂性脑缺血发作或中风病史；植入式心脏复律除颤器（ICD）的使用与死亡呈负相关。排除植入ICD的患者后，与年龄和性别匹配的个体相比生存率无差异（P = 0.39）；年龄、既往短暂性脑缺血发作/中风和终末期HCM是死亡的预测因素。
在该队列中，NO-HCM患者的生存率与年龄和性别匹配的美国白人人群死亡率相似。虽然ICD的使用与死亡呈负相关，但排除这些患者后总体生存率未见差异。终末期HCM与死亡风险增加独立相关，确定了可能从更积极治疗中获益的患者亚组。
