肥厚型心肌病中收缩功能障碍的患病率及临床意义
Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy.
作者信息
Thaman R, Gimeno J R, Murphy R T, Kubo T, Sachdev B, Mogensen J, Elliott P M, McKenna W J
机构信息
The Heart Hospital, University College London, London, UK.
出版信息
Heart. 2005 Jul;91(7):920-5. doi: 10.1136/hrt.2003.031161.
OBJECTIVES
To determine the frequency of systolic impairment (SI) and its impact on the natural history of hypertrophic cardiomyopathy (HCM).
METHODS
1080 patients (mean (SD) age 43 (15) years, 660 men) with HCM were evaluated. Initial assessment included history, examination, 48 hour Holter monitoring, cardiopulmonary exercise testing, and echocardiography; SI was defined as a fractional shortening (FS) < or = 25%. Survival data were collected at clinic visits or by direct communication with patients and their general practitioners. The results of serial echocardiography in 462 patients with normal FS at presentation are also reported.
RESULTS
26 (2.4%) patients (49 (14) years, 18 men) had SI at the initial visit. During follow up (58 (49) months), nine (34.6%) died or underwent cardiac transplantation compared with 108 (10.2%) patients with normal FS (p = 0.01). Five year survival from death (any cause) or transplantation was 90.1% (95% confidence interval (CI) 87.8 to 92.4) in patients with normal systolic function versus 52.4% (95% CI 25.2 to 79.6, p < 0.0001) in patients with SI. In patients who underwent serial echocardiography, 22 (4.8%, aged 41 (15) years) developed SI over 66 (40) months; the annual incidence of SI was 0.87% (95% CI 0.54 to 1.31). On initial evaluation patients who developed SI had a higher frequency of syncope (67 (15.2%) v 10 (45.5%) of those who did not develop SI, p = 0.001), non-sustained ventricular tachycardia (91 (20.6%) v 11 (50%), p = 0.002), and an abnormal blood pressure response on exercise (131 (29.7%) v 15 (68.2%), p = 0.001). Patients with SI had greater wall thinning (p = 0.001), left ventricular cavity enlargement (p < 0.0005), and deterioration in New York Heart Association functional class (p = 0.001) during follow up. Thirteen (59.1%) patients who progressed to SI died or underwent transplantation compared with 38 (8.6%) patients who maintained normal systolic function.
CONCLUSIONS
SI is an infrequent complication of HCM but, when present, is associated with a poor prognosis.
目的
确定收缩功能障碍（SI）的发生率及其对肥厚型心肌病（HCM）自然病程的影响。
方法
对1080例HCM患者（平均（标准差）年龄43（15）岁，男性660例）进行评估。初始评估包括病史、体格检查、48小时动态心电图监测、心肺运动试验和超声心动图检查；SI定义为缩短分数（FS）≤25%。在门诊就诊时或通过与患者及其全科医生直接沟通收集生存数据。还报告了462例初诊时FS正常患者的系列超声心动图检查结果。
结果
26例（2.4%）患者（年龄49（14）岁，男性18例）初诊时存在SI。在随访期间（58（49）个月），9例（34.6%）死亡或接受心脏移植，而FS正常的患者为108例（10.2%）（p = 0.01）。收缩功能正常患者从死亡（任何原因）或移植开始的5年生存率为90.1%（95%置信区间（CI）87.8至92.4），而SI患者为52.4%（95%CI 25.2至79.6，p < 0.0001）。在接受系列超声心动图检查的患者中，22例（4.8%，年龄41（15）岁）在66（40）个月内出现SI；SI的年发生率为0.87%（95%CI 0.54至1.31）。在初始评估时，发生SI的患者晕厥发生率较高（67例（15.2%）对未发生SI的患者10例（45.5%），p = 0.001）、非持续性室性心动过速发生率较高（91例（20.6%）对11例（50%），p = 0.002）以及运动时血压反应异常发生率较高（131例（29.7%）对15例（68.2%），p = 0.001）。SI患者在随访期间心肌变薄更明显（p = 0.001）、左心室腔扩大（p < 0.0005）且纽约心脏协会功能分级恶化（p = 0.001）。进展为SI的13例（59.1%）患者死亡或接受移植，而收缩功能维持正常者为38例（8.6%）。
结论
SI是HCM的一种少见并发症，但一旦出现则预后不良。
Zotero 插件