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肥厚型心肌病(HCM)中的中腔梗阻:一例罕见病例报告及处理方法

Mid-Cavitary Obstruction in Hypertrophic Cardiomyopathy (HCM): A Rare Case Report and Management Approach.

作者信息

Bharadwaj Rajeev, Boruah Deb, Barman Bhupen, Kalita Suman

机构信息

All India Institute of Medical Sciences, Guwahati, India.

出版信息

Acta Med Litu. 2025;32(1):138-144. doi: 10.15388/Amed.2025.32.1.16. Epub 2025 Feb 18.

Abstract

UNLABELLED

Hypertrophic cardiomyopathy (HCM) with mid-cavitary obstruction (MCO) is an uncommon condition affecting approximately 10% of HCM patients which is associated with serious outcomes, including sudden cardiac death and heart failure. We present the case of a 43-year-old male with type 2 diabetes mellitus who experienced worsening dyspnea and palpitations, leading to the diagnosis of HCM with MCO without outlet obstruction. Cardiac MRI with gadolinium contrast reveals significant mid-cavitary obstruction without left ventricular outflow tract obstruction with a scar burden of 27% and the absence of other high-risk factors such as apical aneurysm and NSVT runs on Holter monitoring. The patient was treated conservatively with the oral beta-blocker therapy, resulting in symptomatic improvement. Given the high risk of an adverse outcome, it is crucial to recognise MCO early and provide the appropriate treatment. This case report discusses the presentation, diagnosis, and management of a patient with HCM and mid-cavitary obstruction, highlighting the unique treatment considerations associated with this condition.

TAKE HOME MESSAGE

Mid-cavitary obstruction (MCO) in hypertrophic cardiomyopathy (HCM) is associated with high-risk outcomes of sudden cardiac death and heart failure.While beta-blockers can improve symptoms in many MCO patients, treatment should be personalized based on the symptom severity and risk factors.Patients with MCO are at risk of complications like apical aneurysms, thromboembolism, and arrhythmias.

摘要

未标注

伴有心腔中部梗阻(MCO)的肥厚型心肌病(HCM)是一种罕见病症,约影响10%的HCM患者,与严重后果相关,包括心源性猝死和心力衰竭。我们报告一例43岁2型糖尿病男性患者,其出现进行性加重的呼吸困难和心悸,最终诊断为伴有MCO且无流出道梗阻的HCM。钆增强心脏磁共振成像显示存在显著的心腔中部梗阻,无左心室流出道梗阻,瘢痕负荷为27%,且在动态心电图监测中未发现其他高危因素,如心尖部动脉瘤和非持续性室性心动过速发作。该患者接受口服β受体阻滞剂保守治疗,症状得到改善。鉴于不良后果风险高,早期识别MCO并提供适当治疗至关重要。本病例报告讨论了一名伴有HCM和心腔中部梗阻患者的临床表现、诊断和管理,强调了与该病症相关的独特治疗考量。

要点

肥厚型心肌病(HCM)中的心腔中部梗阻(MCO)与心源性猝死和心力衰竭的高危后果相关。虽然β受体阻滞剂可改善许多MCO患者的症状,但治疗应根据症状严重程度和危险因素进行个体化。MCO患者有发生心尖部动脉瘤、血栓栓塞和心律失常等并发症的风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ddc4/12239168/9d38894bea03/amed-32-138-g001.jpg

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