A Young Male Patient with Subaortic Membranous Stenosis and Left Ventricular NonCompaction Cardiomyopathy: A Case Report

IF 0.5 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Said Abdirahman Ahmed, Mohamed Farah Yusuf Mohamud, Mohamed Abdullahi mohamud
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Abstract

: Left ventricular noncompaction cardiomyopathy (LVNC) is a relatively rare primary genetic cardiomyopathy and increased in the frequency of detection. LVNC is characterized by prominent wall trabeculations and intertrabecular recesses that communi-cate with the ventricular cavity. It could be in isolated form or coexists with other congenital heart diseases including valvular heart disease. The prevalence of adult LVNC ranges from 0.01% to 0.27%. This present case is a 19-year-old male patient who presents as a cardiology outpatient with progressive dyspnea for one month. Physical examination revealed tachycardia and third heart sound on auscultation. A complete left bundle branch block was detected on electrocardiography, and chest X-ray showed an enlarged cardiac shadow (cardiomegaly). Echocardiography revealed left ventricular systolic dysfunction (LVEF: 25%), a noncompact layer, hypertrabeculation, and subaortic membranous stenosis with P-mean of 32 mmHg. The patient had started heart failure management and scheduled cardiac resynchronization therapy (CRT) for life-saving and recommended to search for either left ventricular assist device (LVAD) or heart transplantation where he can. In conclusion, the symptoms of heart failure and cardiac arrhythmias should be considered significant in apparently healthy young patients. Besides, intensive medical treatment has indicated the implantation of cardiac resynchronization therapy (CRT) “life-saving” and advanced cases of heart transplantation.
一例年轻男性皮质下膜狭窄合并左心室非致密性心肌病病例报告
左室非压实性心肌病(LVNC)是一种相对罕见的原发性遗传性心肌病,其检出率呈上升趋势。LVNC的特征是突出的壁小梁和与脑室相通的小梁间窝。它可能是孤立的形式或与其他先天性心脏病共存,包括瓣膜性心脏病。成人LVNC患病率为0.01% ~ 0.27%。本病例是一名19岁男性患者,因进行性呼吸困难就诊一个月。体格检查发现心动过速和第三心音。在心电图上发现完整的左束支阻滞,胸部x线显示心脏阴影增大(心脏肥大)。超声心动图显示左室收缩功能障碍(LVEF: 25%),非致密层,小梁亢进,主动脉下膜性狭窄,p平均值为32 mmHg。患者已经开始心衰管理和计划的心脏再同步化治疗(CRT)以挽救生命,并建议在可能的情况下寻找左心室辅助装置(LVAD)或心脏移植。总之,在表面健康的年轻患者中,心衰和心律失常的症状应被认为是重要的。此外,密集的医学治疗表明,植入心脏再同步化治疗(CRT)“救命”和晚期心脏移植病例。
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来源期刊
Research Reports in Clinical Cardiology
Research Reports in Clinical Cardiology CARDIAC & CARDIOVASCULAR SYSTEMS-
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发文量
11
审稿时长
16 weeks
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