An Shuoyan, Fan Chaomei, Yan Lirong, Cai Chi, Yang Yinjian, Zhai Shanshan, Zhao Shihua, Liu Yanling, Duan Fujian, Wang Zhimin, Li Yishi
Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Cardiology. 2017;136(2):108-114. doi: 10.1159/000448239. Epub 2016 Sep 6.
As reported, diagnostic age, gender and presence of outflow tract obstruction have an impact on prognosis in patients with hypertrophic cardiomyopathy. The aim of this study was to compare the long-term outcome between apical hypertrophic cardiomyopathy (ApHCM) and asymmetric septal hypertrophic cardiomyopathy (ASHCM) after the exclusion of these factors.
A total of 540 patients (270 with ApHCM and 270 with ASHCM) identified in a consecutive single-center cohort were retrospectively studied. The two groups were matched by diagnostic age, gender and the presence of outflow tract obstruction. Clinical characteristics and long-term outcomes were compared.
The mean follow-up duration in ASHCM and ApHCM were 6.6 ± 5.5 and 7.6 ± 4.1 years, respectively. During follow-up, 16 patients experienced cardiovascular death in the ASHCM group, while 2 patients experienced cardiovascular death in the ApHCM group (6.3 vs. 0.7%, p < 0.01). Cardiovascular morbidity in the ASHCM and ApHCM groups were 39.9 and 18.5% (p < 0.01). In the multivariate Cox regression analysis late gadolinium enhancement (LGE; HR 4.81, 95% CI 1.28-78.0, p = 0.03) and unexplained syncope (HR 9.68, 95% CI 1.9-17.2, p < 0.01) were independent predictors for cardiovascular mortality. Unexplained syncope was independently associated with a higher risk for sudden cardiac death (HR 4.3, 95% CI 1.2-15.3, p = 0.02).
After eliminating the interference of diagnostic age, gender and outflow tract obstruction, ASHCM represented a worse prognosis with a higher incidence of cardiovascular mortality and morbidity than ApHCM. LGE was a strong predictor for cardiovascular death.
据报道，诊断年龄、性别及流出道梗阻的存在对肥厚型心肌病患者的预后有影响。本研究的目的是在排除这些因素后，比较心尖肥厚型心肌病（ApHCM）和非对称性室间隔肥厚型心肌病（ASHCM）的长期预后。
对连续入选的单中心队列中的540例患者（270例ApHCM和270例ASHCM）进行回顾性研究。两组在诊断年龄、性别及流出道梗阻存在情况方面进行匹配。比较临床特征和长期预后。
ASHCM组和ApHCM组的平均随访时间分别为6.6±5.5年和7.6±4.1年。随访期间，ASHCM组有16例患者发生心血管死亡，而ApHCM组有2例患者发生心血管死亡（6.3%对0.7%，p<0.01）。ASHCM组和ApHCM组的心血管疾病发病率分别为39.9%和18.5%（p<0.01）。在多因素Cox回归分析中，延迟钆增强（LGE；HR 4.81，95%CI 1.28 - 78.0，p = 0.03）和不明原因晕厥（HR 9.68，95%CI 1.9 - 17.2，p<0.01）是心血管死亡的独立预测因素。不明原因晕厥与心脏性猝死风险较高独立相关（HR 4.3，95%CI 1.2 - 15.3，p = 0.02）。
在消除诊断年龄、性别及流出道梗阻的干扰后，ASHCM的预后较ApHCM更差，心血管死亡率和发病率更高。LGE是心血管死亡的有力预测因素。
