马其顿扩张型左心室非压迫性心肌病患者对心脏再同步化疗法超级反应的罕见病例

Bozhin Shopov, P. Zafirovska, Jorgo Kostov, Nikola Manev, Antonio Georgiev
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摘要

背景:左心室不充盈(LVNC)是一种罕见的心肌病,心内膜小梁增多且突出,又称海绵状心肌。它通常与扩张型心肌病(DCM)同时存在,并且心力衰竭(HF)的发病率很高。目前,心脏再同步化疗法(CRT)被现有指南推荐用于选定的射血分数降低型心力衰竭(HFrEF)患者。目的:我们的病例报告旨在强调左心室非充盈性心肌病和 HFrEF 患者对 CRT 的治疗效果和超级反应:55 岁的马其顿男性患者,患有 HFrEF 和左束支传导阻滞 (LBBB),尽管接受了最佳药物治疗 (OMT),但仍无症状(NYHA III)。超声心动图和CMR检查结果为扩张型和左心室非充盈性心肌病。在植入 CRT-P 设备 18 个月后,我们的心肌收缩功能完全恢复(EF 从 23% 恢复到 53%),左心室内径从 90 毫米缩小到 64 毫米,左心室收缩末期容积(LVESV)从 319 毫升缩小到 98 毫升,患者的生活质量明显改善:心脏再同步化疗法是治疗因左心室非充盈性扩张型心肌病导致的高房颤动先兆流产(HFrEF)患者的一种安全而有价值的方法。
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Rare case of super-response to Cardiac Resynchronization Therapy in Macedonian patient with Dilated Left Ventricular Non-Compaction Cardiomyopathy
Background: Left ventricular non-compaction (LVNC) is rare cardiomyopathy with increased and prominent endomyocardial trabeculations also known as spongy myocardium. It is often found in association with a dilated cardiomyopathy (DCM) and has high incidence of Heart failure (HF). Cardiac resynchronization therapy (CRT) is currently recommended by the available guidelines for selected patients with Heart failure with reduced ejection fraction (HFrEF). Aim: Our case report aims to highlight the therapeutic benefits and superresponse to CRT in a patient with Left ventricular non-compaction cardiomyopathy and HFrEF. Case report: 55-year-old Macedonian male patient with HFrEF, Left bundle branch block (LBBB) remained symptomatic (NYHA III) despite optimal medical treatment (OMT). Echocardiography and CMR findings were in addition to dilated and left ventricular non-compaction cardiomyopathy. Cardiac resynchronization therapy was indicated and 18 months after implantation of CRT-P device we have achieved complete and utter reversibility of systolic myocardial function (EF from 23% to 53%), left ventricular internal diameter was reduced from 90mm to 64mm, left ventricular end systolic volume (LVESV) was reduced from 319ml to 98ml and patient quality of life significantly improved. Conclusion: Cardiac resynchronization therapy is a safe and valuable method of treatment for patients with HFrEF due to dilated left ventricular non-compaction cardiomyopathy.
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