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伴有异常冠状动脉的晚期心力衰竭中的左心室心肌致密化不全:一例报告

Left Ventricular Noncompaction in Advanced Heart Failure With an Anomalous Coronary Artery: A Case Report.

作者信息

Quasem Khaleel, Carrasquel Michelle, Felice Jordan, Smith Britni, Baraka Dania, Khasawneh Karam, Quasem Adam, Mughal Majid

机构信息

Internal Medicine, McLaren Greater Lansing, Lansing, USA.

Cardiology, McLaren Greater Lansing, Lansing, USA.

出版信息

Cureus. 2025 Mar 4;17(3):e80015. doi: 10.7759/cureus.80015. eCollection 2025 Mar.

DOI:10.7759/cureus.80015
PMID:40182365
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11966663/
Abstract

Left ventricular noncompaction (LVNC) involves abnormal development of the heart muscle, where the inner layer remains excessively trabeculated instead of compacting properly. Traditionally considered congenital, increasing reports of nonfamilial or sporadic LVNC suggest that adverse myocardial remodeling, such as from volume overload or preexisting cardiomyopathy, may contribute to its development. We present the case of a 77-year-old male with chronic atrial fibrillation and nonischemic cardiomyopathy who was found to have severe LVNC, identified on echocardiography and cardiac MRI using a noncompacted-to-compacted myocardial thickness ratio >2.3 at end-systole. Severe left ventricular dysfunction (ejection fraction (EF) <20%) was confirmed, and angiography revealed nonobstructive coronary disease with an anomalous left circumflex artery - an uncommon co-occurrence that underscores the importance of comprehensive imaging. The late presentation of LVNC in an older adult without a familial history expands the recognized demographic and suggests that structural and hemodynamic stressors may play a role in its development. Management included pharmacologic rate control for atrial fibrillation, electrical cardioversion, and guideline-directed therapy for heart failure with reduced EF, ultimately leading to implantable cardioverter-defibrillator placement for primary prevention of sudden cardiac death. Following these interventions, the patient showed modest improvement in functional status and symptoms and remains under close follow-up for device surveillance and serial imaging. This case broadens our understanding of LVNC by highlighting its potential for late onset, the necessity of multimodality imaging to detect coexisting anomalies, and the importance of a comprehensive treatment approach to optimize outcomes in older adults.

摘要

左心室心肌致密化不全(LVNC)涉及心肌的异常发育,其内层保持过度的小梁化,而不是正常致密化。传统上认为是先天性的,但越来越多关于非家族性或散发性LVNC的报道表明,诸如容量超负荷或既往存在的心肌病等不良心肌重塑可能促成其发生发展。我们报告一例77岁男性,患有慢性心房颤动和非缺血性心肌病,经超声心动图和心脏磁共振成像检查发现患有严重LVNC,采用收缩末期非致密化与致密化心肌厚度比>2.3来确定。已证实存在严重的左心室功能障碍(射血分数(EF)<20%),血管造影显示非阻塞性冠状动脉疾病伴左旋支动脉异常——这种罕见的共存情况凸显了综合成像的重要性。在无家族史的老年患者中LVNC的晚期表现扩大了已认识的人群范围,并提示结构和血流动力学应激因素可能在其发生发展中起作用。治疗包括针对心房颤动的药物心率控制、电复律以及针对射血分数降低的心力衰竭的指南指导治疗,最终导致植入式心律转复除颤器植入以一级预防心脏性猝死。经过这些干预措施后,患者的功能状态和症状有适度改善,目前仍在密切随访以进行设备监测和系列成像检查。该病例通过强调其晚发的可能性、多模态成像检测共存异常的必要性以及全面治疗方法对优化老年患者预后的重要性,拓宽了我们对LVNC的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/2f8eab65e1b6/cureus-0017-00000080015-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/a2848daf7bc7/cureus-0017-00000080015-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/ece6dcce5037/cureus-0017-00000080015-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/2f8eab65e1b6/cureus-0017-00000080015-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/a2848daf7bc7/cureus-0017-00000080015-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/ece6dcce5037/cureus-0017-00000080015-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/83fd/11966663/2f8eab65e1b6/cureus-0017-00000080015-i03.jpg

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