Frey Simon M, Hofmann Verena, Zellweger Michael J, Haaf Philip
Department of Cardiology, Cardiovascular Research Institute Basel (CRIB), University Hospital Basel, University of Basel, Petersgraben 4, CH-4031 Basel, Switzerland.
Department of Radiology, University Hospital Basel, University of Basel, Basel, Switzerland.
Eur Heart J Case Rep. 2021 Sep 24;5(10):ytab380. doi: 10.1093/ehjcr/ytab380. eCollection 2021 Oct.
Mitral annular calcification (MAC) is a degenerative, mostly asymptomatic abnormality usually in elderly patients. Caseous MAC (cMAC) is a rare form with central liquefaction necrosis, which typically involves the posterior annulus of the mitral valve and can cause serious sequelae. However, optimal management of patients with cMAC is not clearly defined.
In a 71-year-old female patient, MAC was incidentally detected. Tissue characterization with cardiac magnetic resonance (CMR) revealed a cMAC and a conservative approach was chosen. Six months after cMAC diagnosis, the patient developed an acute hemi-occlusion of a retinal artery with cholesterol embolism. At this time, CMR showed a liquefied cavity of the cMAC. Except for atherosclerotic plaques in the aorta and carotid arteries, further stroke work-up was negative. Therefore, the conservative approach was continued. During follow-up, the liquefied cavity regressed completely after another 6 months and the patient was free from further events (total follow-up 3 years since diagnosis of cMAC).
A clear diagnosis and quantitative assessment of dynamic processes, such as cMAC, are made possible by performing CMR with multi-parametric tissue characterization. Dynamic changes in cMAC may have serious clinical implications, such as mitral regurgitation or systemic embolization. Among cardiac tumours, thrombus and abscess, cMAC should be included in the differential diagnosis of an intracardiac mass of the posterior mitral annulus in order to avoid further inappropriate diagnostic interventions.
二尖瓣环钙化(MAC)是一种退行性病变,多见于老年患者,通常无症状。干酪样二尖瓣环钙化(cMAC)是一种罕见形式,伴有中央液化坏死,通常累及二尖瓣后瓣环,可导致严重后果。然而,cMAC患者的最佳治疗方案尚无明确定义。
一名71岁女性患者偶然发现有MAC。心脏磁共振成像(CMR)的组织特征显示为cMAC,遂选择保守治疗方法。cMAC诊断6个月后,患者发生视网膜动脉急性半闭塞并伴有胆固醇栓塞。此时,CMR显示cMAC有液化腔。除主动脉和颈动脉有动脉粥样硬化斑块外,进一步的卒中检查结果为阴性。因此,继续采用保守治疗方法。在随访期间,液化腔在又过了6个月后完全消退,患者未再发生其他事件(自cMAC诊断以来总随访3年)。
通过多参数组织特征的CMR检查,可以对cMAC等动态过程进行明确诊断和定量评估。cMAC的动态变化可能具有严重的临床意义,如二尖瓣反流或全身栓塞。在心脏肿瘤、血栓和脓肿的鉴别诊断中,二尖瓣后瓣环心内肿块的鉴别诊断应包括cMAC,以避免进一步不适当的诊断干预。