心尖肥厚型心肌病与典型肥厚型心肌病的临床特点比较

Comparison of cfinical characteristics of Chinese patients with apical hypertrophic cardiomyopathyand typical hypertrophic cardiomyopathy

目的通过与典型的肥厚型心肌病（HCM）比较，探讨中国汉族人心尖肥厚型心肌病（AHCM）的临床特征及其诊治方法。方法回顾性收集160例HCM住院患者的临床资料，分成3组进行比较。AHCM组：AHCM41例。典型HCM患者（室间隔以及左心室壁肥厚），根据是否存在流出道梗阻分成2组，即非梗阻性肥厚型心肌病（NOHCM）组，52例，左心室流出道压差〈30mmHg（1mmHg=0．133kPa）；梗阻性肥厚型心肌病（OHCM）组，67例，左心室流出道压差≥30mmHg。比较3组患者的临床症状、诊治方法以及血浆生物标记物水平。结果（1）AHCM组患者的发病年龄较OHCM组晚[（49．94±13．6）岁比（41．4±14．6）岁，P〈0．01]，无猝死家族史，较少出现劳力性呼吸困难，血浆血N末端B型利钠肽原（NT-proBNP）水平较OHCM组低（P〈0．01）。血浆心肌酶中肌酸激酶同工酶（CK-MB）、乳酸脱氢酶（LDH）、肌钙蛋白I（TnI）和肌红蛋白（MYO）的水平在3组间差异均无统计学意义。（2）31例AHCM患者因冠心病收治入院，经检查后，18例（18／41，43．9％）排除了冠心病。（3）AHCM组、NOHCM组和OHCM组心电图上深尖倒置T波（GNT）出现的频率分别为43．9％、13．5％和4．4％（P〈0．01），且AHCM组多伴有左心室高电压。（4）心脏核磁共振诊断AHCM明显优于心脏超声，与诊断典型HCM相比更有优势。结论AHCM与典型OHCM的临床特点比较差异有统计学意义，而与典型的NOHCM比较差异无统计学意义。心脏核磁共振检查阳性及心电图胸导联上典型的GNT可为确诊AHCM提供依据。

Objective To evaluate the clinical features in Chinese patients with apical hypertrophic cardiomyopathy （AHCM） and typical hypertrophic cardiomyopathy （HCM）. Methods This retrospective analysis included 160 patients hospitalized in Fuwai hospital. Patients were divided into three groups： apical hypertrophic cardiomyopathy （ AHCM, n = 41 ） group, non-obstructive typical hypertrophic cardiomyopathy group[NOHCM, LVOT 〈 30 mm Hg（ 1 mm Hg = 0. 133 kPa） at rest, n = 52] and obstructive typical hypertrophic cardiomyopathy （OHCM, LVOT ≥30 mm Hg at rest, n = 67 ）. Clinical features, diagnosis, therapy, and plasma levels of biomarkers of these three groups were analyzed. Results （ 1 ） The age at disease onset was older in AHCM group than in OHCM group [ （49. 9 ± 13.6） years vs. （41.4 ± 14.6） years, P 〈 0.01 ]. Exertional dyspnea appered more often in HCM patients than in AHCM patients, NTproBNP level was significantly lower in AHCM patients than in OHCM patients （ P = 0. 001 ）. Plasma CK- MB, LDH, TnI and MYO levels were similar among the three groups. （2） Thirty-three AHCM patients were first hospitalized for suspected coronary heart disease （CHD） and CHD was excluded in 18 cases （43. 9% ）. （3） The frequency of giant negative T waves （depth I〉 10 ram） on ECG was 43.9%, 13.5% and 4. 4%（P 〈0. 01） in AI-ICM, NOHCM and OHCM respectively. Half of AHCM patients showed left ventricular high voltage on ECG. （4） Cardiac magnetic resonance imaging is superior to echocardiography on correctly diagnosing AHCM. Conclusion AHCM patients differ from typical OHCM patients in clinical characteristics. There were significant differences on echocardiography and electrocardiography features among three groups. Cardiac magnetic resonance imaging and giant negative T waves on ECG are helpful for the diagaosis of AHCM.