产妇死亡和孤立性左室非压实性心肌病

IF 0.3 Q3 MEDICINE, GENERAL & INTERNAL
R. Rahimi, M. Shahidan, K. Thanikasalam
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引用次数: 0

摘要

摘要左心室非压实性心肌病(LVNC)是一种罕见的先天性心肌病,其特征是左心室的超小窝和深凹。患者可能无症状或有常见表现,包括收缩功能降低、心律失常、血栓栓塞事件和心力衰竭。这种情况的罕见性以及缺乏适当的评估可能导致这种情况在很大程度上未被诊断或未被认识。一位23岁的女士在生完第一个孩子31天后在家中昏倒。她在一年前被诊断出甲状腺肿,并在整个怀孕期间表现良好。尸检结果显示左心室小梁增加。手术后获得了进一步的病史,显示晕厥发作和双侧下肢无力等症状可追溯到她突然死亡前5年。这些特征与左心室功能障碍导致的收缩功能降低和射血分数降低所导致的低血压低灌注效应一致。虽然LVNC仍然是一种罕见的疾病,但我们想强调良好记忆的重要性。它可能有助于发现一些罕见的病理,如这种心脏疾病,从而保证适当的心脏成像,以确定初步诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Maternal death and isolated left ventricular non-compaction cardiomyopathy
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital cardiomyopathy, which is characterized by hypertrabeculations and deep recesses of the left ventricle. A patient could be asymptomatic or presented with common manifestations, including reduced systolic function, arrhythmia, thromboembolic events and heart failure. The rarity of the condition as well as lack of proper assessment has probably led to this condition to be largely underdiagnosed or unrecognized. A 23-year-old lady had collapsed at home thirty one days after delivering her first child. She had a history of goitre diagnosed a year ago and noted to be fairly well throughout the pregnancy. Post mortem findings showed increased trabeculations of the left ventricle. Further history was obtained after the procedure, revealing symptoms such as syncopal attacks and bilateral lower limb weakness dated back as far as five years prior to her sudden demise. These features were in keeping with hypotension hypoperfusion effects resulted from reduced systolic function and decreased ejection fraction, as a result of left ventricular dysfunction. While LVNC remains a rare type of disease, we would like to highlight the importance of a good anamnesis. It may help to uncover some uncommon pathology such as this heart disease, thus warranting an appropriate cardiac imaging to be engaged to clinch the primary diagnosis.
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来源期刊
International Medical Journal Malaysia
International Medical Journal Malaysia Medicine-Medicine (all)
CiteScore
0.40
自引率
0.00%
发文量
73
期刊介绍: International Medical Journal Malaysia (IMJM) is the official journal of the Kulliyyah (Faculty) of Medicine, International Islamic University Malaysia. It serves primarily as a forum for education and intellectual discourse for health professionals namely in clinical medicine but covers diverse issues relating to medical ethics, professionalism as well as medical developments and research in basic medical sciences. It also serves the unique purpose of highlighting issues and research pertaining to the Muslim world. Contributions to the IMJM reflect its international and multidisciplinary readership and include current thinking across a range of specialties, ethnicities and societies.
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