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超越常规:右位心患者的心肌致密化不全、室性心动过速和收缩功能障碍:病例系列

Beyond convention: non-compacted myocardium, ventricular tachycardia, and systolic dysfunction in dextrocardia patients: a case series.

作者信息

Nazarenko Natalia, Maliha Maisha, Cerna Luis, Abittan Nathaniel, Borkowski Pawel, Csecs Ibolya, Garcia Mario

机构信息

Department of Medicine, Jacobi Medical Center/AECOM, Bronx, New York.

Department of Medicine, Dhaka Medical College, Dhaka, Bangladesh.

出版信息

Ann Med Surg (Lond). 2025 Jan 9;87(1):56-61. doi: 10.1097/MS9.0000000000002855. eCollection 2025 Jan.

Abstract

BACKGROUND

Noncompaction of the left ventricle (LVNC) is linked to a higher risk of sudden cardiac death and stroke. Its prevalence ranges from 0.014% to 1.3% in the general population, rising to 7.5% in patients with dextrocardia.

CASE SUMMARY

A male in his late 70s presented with worsening dyspnea and leg swelling, with dextrocardia and frequent extrasystoles. Imaging revealed right-sided pleural effusion, severely reduced ejection fraction, and ventricular and atrial dilatation. He developed sustained monomorphic VT, was treated with amiodarone, had successful coronary stenting, and received an ICD with no further hospital readmissions or ICD events. A second case involved a male in his late 50s who presented with dyspnea. He had dextrocardia with situs inversus, subsegmental pulmonary embolism, and LVNC. He was treated with enoxaparin, medical therapy, and Holter monitoring, which showed mild arrhythmias. He declined ICD placement but remained event-free during the first year of follow-up.

DISCUSSION

LVNC is a rare condition resulting from abnormal myocardial development during embryogenesis, leading to a two-layered myocardial structure. Diagnosis is based on imaging criteria. LVNC is linked to arrhythmias, heart failure, and conduction abnormalities, requiring interventions such as ICD placement, arrhythmia monitoring, and genetic testing. Further research is needed on genetic associations and long-term outcomes in dextrocardia patients.

摘要

背景

左心室心肌致密化不全(LVNC)与心脏性猝死和中风的较高风险相关。其在普通人群中的患病率为0.014%至1.3%,在右位心患者中升至7.5%。

病例摘要

一名70多岁的男性因呼吸困难和腿部肿胀加重就诊,患有右位心和频发早搏。影像学检查显示右侧胸腔积液、射血分数严重降低以及心室和心房扩大。他发生了持续性单形性室性心动过速,接受了胺碘酮治疗,成功进行了冠状动脉支架置入术,并植入了植入式心律转复除颤器(ICD),此后未再住院或发生ICD相关事件。第二例患者是一名50多岁的男性,因呼吸困难就诊。他患有右位心伴内脏转位、节段性肺栓塞和LVNC。他接受了依诺肝素治疗、药物治疗和动态心电图监测,结果显示有轻度心律失常。他拒绝植入ICD,但在随访的第一年未发生任何事件。

讨论

LVNC是一种罕见疾病,由胚胎发育过程中心肌异常发育导致心肌结构分为两层。诊断基于影像学标准。LVNC与心律失常、心力衰竭和传导异常有关,需要进行如植入ICD、心律失常监测和基因检测等干预措施。对于右位心患者的基因关联和长期预后,还需要进一步研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7aa/11918691/80bcf4810da6/ms9-87-056-g001.jpg

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