Finsterer Josef, Zarrouk-Mahjoub Sinda
Josef Finsterer, Krankenanstalt Rudolfstiftung, 1030 Vienna, Austria.
World J Cardiol. 2016 May 26;8(5):333-9. doi: 10.4330/wjc.v8.i5.333.
Mitochondrial disorders (MIDs) are usually multisystem disorders (mitochondrial multiorgan disorder syndrome) either on from onset or starting at a point during the disease course. Most frequently affected tissues are those with a high oxygen demand such as the central nervous system, the muscle, endocrine glands, or the myocardium. Recently, it has been shown that rarely also the arteries may be affected (mitochondrial arteriopathy). This review focuses on the type, diagnosis, and treatment of mitochondrial vasculopathy in MID patients. A literature search using appropriate search terms was carried out. Mitochondrial vasculopathy manifests as either microangiopathy or macroangiopathy. Clinical manifestations of mitochondrial microangiopathy include leukoencephalopathy, migraine-like headache, stroke-like episodes, or peripheral retinopathy. Mitochondrial macroangiopathy manifests as atherosclerosis, ectasia of arteries, aneurysm formation, dissection, or spontaneous rupture of arteries. The diagnosis relies on the documentation and confirmation of the mitochondrial metabolic defect or the genetic cause after exclusion of non-MID causes. Treatment is not at variance compared to treatment of vasculopathy due to non-MID causes. Mitochondrial vasculopathy exists and manifests as micro- or macroangiopathy. Diagnosing mitochondrial vasculopathy is crucial since appropriate treatment may prevent from severe complications.
线粒体疾病(MIDs)通常为多系统疾病(线粒体多器官障碍综合征),要么起病时即为多系统受累,要么在病程中的某个时间点开始累及多个系统。最常受累的组织是那些对氧气需求较高的组织,如中枢神经系统、肌肉、内分泌腺或心肌。最近有研究表明,动脉也可能很少受到影响(线粒体动脉病变)。本综述重点关注MID患者中线粒体血管病变的类型、诊断和治疗。我们使用适当的检索词进行了文献检索。线粒体血管病变表现为微血管病变或大血管病变。线粒体微血管病变的临床表现包括白质脑病、偏头痛样头痛、类中风发作或周边视网膜病变。线粒体大血管病变表现为动脉粥样硬化、动脉扩张、动脉瘤形成、动脉夹层或动脉自发性破裂。诊断依赖于排除非MID病因后,对线粒体代谢缺陷或遗传病因的记录和确认。与非MID病因导致的血管病变的治疗相比,其治疗并无差异。线粒体血管病变确实存在,并表现为微血管病变或大血管病变。诊断线粒体血管病变至关重要,因为恰当的治疗可预防严重并发症。