Kusukawa J, Suwa M, Nakayama Y, Hirota Y, Kawamura K, Adachi I, Akagi H
Third Division of Internal Medicine, Osaka Medical College, Takatsuki.
J Cardiol. 1988 Mar;18(1):259-69.
In view of the paucity of reports describing symptoms of increased degree, and deterioration of left ventricular systolic function in patients with apical hypertrophic cardiomyopathy (apical HCM), two cases with congestive heart failure and progressive thinning of previously hypertrophied apical portions of the left ventricle are reported. These were among 13 patients observed from eight to 10 years. Case 1: A 56-year-old man was diagnosed as having apical HCM at the age of 49 years. Severe left ventricular hypertrophy and prominent ST-T changes were observed on ECG during his first admission. His left ventricular end-diastolic pressure (LVEDP) was 24 mmHg and a left ventriculo-gram revealed a decrease in the left ventricular cavity in the apex and marked hypertrophy of the apical wall. Moderate interstitial fibrosis without hypertrophy or disarray of myocytes was observed in a left ventricular endomyocardial biopsy specimen. In two episodes of cardiac arrest he was successfully resuscitated at the age of 50 years. At the age of 55 years, two-dimensional echocardiography revealed thinning and abnormal motion in the apical wall, and a defect in 201T1 accumulation was observed in the same region by perfusion scintigraphy. This patient was readmitted with a diagnosis of cerebral embolism at the age of 56 years. Cardiac catheterization revealed normal LVEDP (8 mmHg), and a left ventriculogram revealed an aneurysm in the left ventricular apex with normal major epicardial coronary arteries. He has been under treatment with antiarrhythmic medications, calcium antagonists and anticoagulants, and has become relatively asymptomatic. Case 2: A 69-year-old-man was diagnosed as having apical HCM after a complete evaluation, including cardiac catheterization, at the age of 59 years. His LVEDP was elevated (17 mmHg), and a left ventricular angiogram revealed marked hypertrophy localized to the apex. Ejection fraction was 64%. A left ventricular endomyocardial biopsy revealed interstitial fibrosis without hypertrophy of myocytes. Thereafter, he has been followed as a New York Heart Association functional class III to IV with occasional elevation of cardiac enzymes but without chest pain or acute changes in his ECGs. However, atrial fibrillation with complete right bundle branch block developed at the age of 60 years. Apical wall thinning and dyskinesis were diagnosed by 2D echocardiography and a defect in the 201T1 accumulation was observed at about 65 years of age. He was readmitted in severe cardiac failure at the age of 69 years, and he was diagnosed as having cardiac asthma with pulmonary capillary wedge pressure of 35 mmHg.(ABSTRACT TRUNCATED AT 400 WORDS)
鉴于关于心尖肥厚型心肌病（apical HCM）患者症状加重程度及左心室收缩功能恶化的报道较少，本文报告了两例充血性心力衰竭且之前肥厚的心尖部左心室逐渐变薄的病例。这两例患者是在观察的13例患者中随访8至10年的病例。病例1：一名56岁男性在49岁时被诊断为心尖HCM。首次入院时心电图显示严重左心室肥厚及明显的ST-T改变。其左心室舒张末压（LVEDP）为24 mmHg，左心室造影显示心尖部左心室腔减小，心尖壁明显肥厚。左心室心内膜活检标本显示中度间质纤维化，未见心肌细胞肥大或排列紊乱。他在50岁时发生两次心脏骤停，均成功复苏。55岁时，二维超声心动图显示心尖壁变薄及运动异常，灌注闪烁显像显示同一区域201T1摄取缺损。该患者56岁时因脑栓塞再次入院。心导管检查显示LVEDP正常（8 mmHg），左心室造影显示左心室心尖部有动脉瘤，主要心外膜冠状动脉正常。他一直在接受抗心律失常药物、钙拮抗剂和抗凝剂治疗，目前相对无症状。病例2：一名69岁男性在59岁时经包括心导管检查在内的全面评估后被诊断为心尖HCM。其LVEDP升高（17 mmHg），左心室造影显示心尖部明显肥厚。射血分数为64%。左心室心内膜活检显示间质纤维化，未见心肌细胞肥大。此后，他一直按照纽约心脏协会心功能Ⅲ至Ⅳ级进行随访，心肌酶偶尔升高，但无胸痛或心电图急性改变。然而，他在60岁时发生房颤并伴有完全性右束支传导阻滞。65岁左右二维超声心动图诊断为心尖壁变薄及运动障碍，201T1摄取缺损。他69岁时因严重心力衰竭再次入院，诊断为心源性哮喘，肺毛细血管楔压为35 mmHg。（摘要截取自400字）
