A case of mid-apical obstructive hypertrophic cardiomyopathy treated with a transapical myectomy approach: a case report.
A case of mid-apical obstructive hypertrophic cardiomyopathy treated with a transapical myectomy approach: a case report.
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DOI：
10.1186/1752-1947-8-364
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发表时间：
2014-11-11
影响因子：
1
通讯作者：
Hueb W
中科院分区：
文献类型：
--
作者：
Scudeler TL;Rezende PC;Oikawa FT;da Costa LM;Hueb AC;Hueb W
肥厚型心肌病是一种遗传性心脏病，其特征是在形态学表达和自然史上具有显著的变异性。肥厚心肌通常局限于左心室的间隔或侧壁，但也可发生在心肌的中段或心尖段。治疗以药物治疗为基础。其他治疗，如中隔动脉栓塞或心室肌切除术，适用于特殊情况。手术是标准治疗方法，通常通过经主动脉入路进行;然而，在肥厚心肌局限于心尖中段的情况下，经心尖入路是一种选择。在英文文献中，只有少数病例报告了采用经心尖入路行肌切除术治疗心尖中段梗阻性肥厚型心肌病。在这份报告中，我们提出了一个病例与中间心尖梗阻性肥厚型心肌病使用这种新的方法治疗。一名63岁的白人女性，有胸痛和呼吸急促病史，导致其日常生活活动严重受限。她有冠状动脉疾病史。她的体格检查无异常。经胸超声心动图显示正常的收缩功能和显著的向心性左心室肥厚，在心尖中部区域更大。核磁共振成像证实左心室中段显著肥大。尽管接受了优化的药物治疗，但患者症状持续存在，需要手术入路。由于认为在该病例中难以使用经主动脉技术进行肌切除术，因此使用了经心尖入路。没有发生并发症，症状得到缓解。对于药物治疗无效的心尖中段梗阻性肥厚型心肌病患者，应考虑经心尖肌切除术。
Hypertrophic cardiomyopathy is a genetic cardiac disease characterized by marked variability in morphological expression and natural history. The hypertrophic myocardium is often confined to the septum or lateral wall of the left ventricle, but it can also be encountered in the middle or apical segments of the myocardium. Treatment is based on medical therapy. Others therapies, such as embolization of the septal artery or ventriculomyectomy, are indicated in special situations. Surgery is the standard treatment, and it is classically done via a transaortic approach; however, in cases in which the hypertrophic myocardium is confined to mid-apical segments, a transapical approach is an option. Only a few cases of mid-apical obstructive hypertrophic cardiomyopathy treated with a myectomy using a transapical approach have been reported in the English-language literature. In this report, we present a case of a patient with mid-apical obstructive hypertrophic cardiomyopathy treated using this new approach. A 63-year-old Caucasian woman presented with a history of chest pain and shortness of breath causing significant limitations on her daily life activities. She had a history of coronary artery disease. Her physical examination was unremarkable. Transthoracic echocardiography revealed normal systolic function and significant concentric left ventricular hypertrophy that was greater in the mid-apical region. Nuclear magnetic resonance imaging confirmed significant hypertrophy of the median segments of the left ventricle. The patient had persistent symptoms despite receiving optimized medical treatment, and a surgical approach was indicated. As a myectomy using transaortic technique was thought to be difficult to perform in her case, a transapical approach was used. No complications occurred, and her symptoms resolved. A transapical myectomy should be taken into consideration for patients with mid-apical obstructive hypertrophic cardiomyopathy that is refractory to medical treatment.