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左心室心肌致密化不全心肌病:沙特阿拉伯1例罕见病例报告。

Left Ventricular Non-compaction Cardiomyopathy: A Report of a Rare Case From Saudi Arabia.

作者信息

Irfan Thalib Husna, Mehveen Sayeeda, Khan Sariya, Haidar Shyma, Jamal Ayesha, Shaikh Ayesha, Alfaqih Mohammed A, Mansy Amir A

机构信息

General Medicine and Surgery, Batterjee Medical College, Jeddah, SAU.

Genetics, St. Ann's College for Women, Hyderabad, IND.

出版信息

Cureus. 2024 Jul 19;16(7):e64937. doi: 10.7759/cureus.64937. eCollection 2024 Jul.

DOI:10.7759/cureus.64937
PMID:39156441
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11330660/
Abstract

Left ventricular non-compaction cardiomyopathy (LVNC) is an unusual congenital heart disease that predominantly affects the heart's left ventricle. This disease is characterized by deep intertrabecular recesses and hypertrabeculations of the myocardial wall that link with the ventricle cavity. During embryogenesis, the fetal myocardium has to undergo a compaction process, wherein the trabeculated and spongy myocardial tissue compacts into a dense, solid form. An incomplete compaction process results in persistent non-compacted spongy myocardial tissue and trabeculations prominent in the left ventricle. This disease could be marked alone or be present in coexistence with other congenital heart abnormalities. We present a rare case of a 57-year-old Saudi male who presented to the ER with chest pain and dyspnea. Due to severe chest pain, he was admitted to the coronary care unit. On further investigation, an echocardiogram revealed heavy trabeculations in the dilated left ventricle and a reduced ejection fraction. The case was diagnosed as LVNC and possible heart failure. The patient was discharged after he was kept under guideline-directed medical therapy (GDMT) along with certain medications and will be evaluated after six months of GDMT to decide on implantable cardiac resynchronization therapy. Although LVNC is rare, it can lead to severe heart conditions like arrhythmias, thromboembolism, and heart failure.

摘要

左心室致密化不全心肌病(LVNC)是一种罕见的先天性心脏病,主要影响心脏的左心室。这种疾病的特征是心肌壁内有深陷的小梁间隙和粗大的小梁,这些小梁与心室腔相连。在胚胎发育过程中,胎儿心肌必须经历一个致密化过程,在此过程中,有小梁的海绵状心肌组织会致密化为致密的固体形式。致密化过程不完全会导致左心室持续存在未致密化的海绵状心肌组织和突出的小梁。这种疾病可以单独出现,也可以与其他先天性心脏异常并存。我们报告一例罕见病例,一名57岁的沙特男性因胸痛和呼吸困难就诊于急诊室。由于严重胸痛,他被收治入冠心病监护病房。进一步检查发现,超声心动图显示扩张的左心室有粗大的小梁,射血分数降低。该病例被诊断为LVNC并可能伴有心力衰竭。患者在接受指南指导的药物治疗(GDMT)并服用某些药物后出院,将在GDMT治疗六个月后进行评估,以决定是否进行植入式心脏再同步治疗。尽管LVNC很罕见,但它可导致严重的心脏疾病,如心律失常、血栓栓塞和心力衰竭。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/8cae1e55308e/cureus-0016-00000064937-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/61348a771607/cureus-0016-00000064937-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/ebca9418b422/cureus-0016-00000064937-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/f54aba4643f2/cureus-0016-00000064937-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/2a2eb231ef83/cureus-0016-00000064937-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/8cae1e55308e/cureus-0016-00000064937-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/61348a771607/cureus-0016-00000064937-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/ebca9418b422/cureus-0016-00000064937-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/f54aba4643f2/cureus-0016-00000064937-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/2a2eb231ef83/cureus-0016-00000064937-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95a/11330660/8cae1e55308e/cureus-0016-00000064937-i05.jpg

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本文引用的文献

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J Saudi Heart Assoc. 2023 Oct 23;35(3):254-257. doi: 10.37616/2212-5043.1352. eCollection 2023.
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Left Ventricular Noncompaction Cardiomyopathy in an Elderly Patient: A Case Report and Literature Review.老年患者左心室心肌致密化不全心肌病:一例报告及文献复习
Cureus. 2023 Apr 29;15(4):e38305. doi: 10.7759/cureus.38305. eCollection 2023 Apr.
3
A Rare Case of Isolated Right Ventricular Non-compaction With the Novel Mutation.
一例伴有新型突变的孤立性右心室心肌致密化不全罕见病例。
Front Cardiovasc Med. 2022 Apr 29;9:845973. doi: 10.3389/fcvm.2022.845973. eCollection 2022.
4
Left ventricular noncompaction cardiomyopathy: Recent advances.左心室致密化不全心肌病:最新进展
Kardiol Pol. 2022;80(5):529-539. doi: 10.33963/KP.a2022.0089. Epub 2022 Apr 2.
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Ventricular non-compaction review.心室非致密化综述。
Heart Fail Rev. 2022 Jul;27(4):1063-1076. doi: 10.1007/s10741-021-10128-3. Epub 2021 Jul 7.
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Noncompaction Cardiomyopathy-History and Current Knowledge for Clinical Practice.非致密型心肌病——临床实践的历史与当前认知
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Non-compaction of ventricular myocardium with polycystic kidney disease with cardiogenic cerebral embolism.伴有心源性脑栓塞的多囊肾病合并心室心肌致密化不全。
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