Olivotto Iacopo, Cecchi Franco, Bini Roberta, Favilli Silvia, Murzi Bruno, El-Hamamsy Ismail, Yacoub Magdi H
Referral Center for Cardiomyopathies, Cardiology Department, Careggi University Hospital, Florence, Italy.
Nat Clin Pract Cardiovasc Med. 2009 Feb;6(2):134-9. doi: 10.1038/ncpcardio1420. Epub 2008 Dec 17.
A 10-year-old boy presented with a history of severe angina on exertion. A two-dimensional echocardiogram showed mild asymmetric left ventricular (LV) hypertrophy localized to the interventricular septum, consistent with nonobstructive hypertrophic cardiomyopathy. A maximal treadmill exercise test was terminated early owing to marked downsloping of the ST-T segment on all precordial leads, associated with mild chest discomfort. Cardiac MRI and coronary angiography showed that the left anterior descending (LAD) artery was 'tunneled' from its origin to the junction of the middle and lower segments, causing systolic obliteration. PET showed diffusely blunted myocardial blood flow after dipyridamole infusion. A beating-heart technique was used to perform surgical mobilization of the superficial and lateral surfaces of the LAD artery. The patient was free from angina at 6 months after surgery. A repeat exercise test showed considerable improvement in exercise tolerance, which was associated with a marked decrease in ST-T changes on exertion.
Physical examination, laboratory tests, 12-lead electrocardiography, two-dimensional echocardiography, exercise testing, cardiac MRI, coronary angiography, PET, Holter electrocardiographic monitoring.
Angina caused by extensive myocardial tunneling of the LAD artery in nonobstructive hypertrophic cardiomyopathy.
Bisoprolol therapy and surgical mobilization of the tunneled LAD artery.
一名10岁男孩有劳力性重度心绞痛病史。二维超声心动图显示左心室（LV）轻度不对称肥厚，局限于室间隔，符合非梗阻性肥厚型心肌病。由于所有胸前导联ST-T段明显下斜，伴有轻度胸痛不适，最大平板运动试验提前终止。心脏磁共振成像和冠状动脉造影显示，左前降支（LAD）动脉从起源至中下段交界处呈“隧道样”，导致收缩期闭塞。双嘧达莫注射后PET显示心肌血流弥漫性减弱。采用不停跳技术对LAD动脉的浅面和侧面进行手术游离。患者术后6个月无心绞痛。重复运动试验显示运动耐量有显著改善，这与运动时ST-T改变明显减少有关。
体格检查、实验室检查、12导联心电图、二维超声心动图、运动试验、心脏磁共振成像、冠状动脉造影、PET、动态心电图监测。
非梗阻性肥厚型心肌病中LAD动脉广泛心肌隧道样改变所致心绞痛。
比索洛尔治疗及对隧道样改变的LAD动脉进行手术游离。
