A Case Series on Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm

Philippe Ryan K. Chung, Emma Y. Gaspar-Trinidad, T. Razon-Cuenza, L. Cuenza
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Abstract

Midventricular obstruction (MVO) is a rare phenotypic subvariant of hypertrophic cardiomyopathy (HCM). It occurs in 1-10% of patients and due to its rarity, its clinical progression and natural history has not yet been extensively studied. However, the available data has shown it to have worse clinical outcomes and prognosis. While reports on hypertrophic cardiomyopathy has been published in the Philippines, there has been no data on this particular variant in the local literature. We identified 3 cases of hypertrophic cardiomyopathy with midventricular obstruction. 2 cases were initially managed as a case of ischemic heart disease who were ruled out after undergoing cardiac magnetic resonance. One case was initially admitted due to a stroke however, an incidental finding on 2D Echo noted the presence of HCM. He also underwent cardiac magnetic resonance which revealed the full extent of his disease. All 3 patients were also found to have apical aneurysms due to ischemia from the pressure coming from the obstruction. Hypertrophic cardiomyopathy is the most common genetic cardiovascular disease but its subvariants are rare and difficult to diagnose. Apical aneurysms are also often missed on 2D Echo. However, cardiac magnetic resonance provides increased detection of the disease with better detailing of the extent and severity. It could also provide information regarding prognostication with regards to arrhythmias and sudden cardiac death. More data however is needed so new avenues for research are open on its application.
肥厚性心肌病合并室中梗阻及顶动脉瘤的病例分析
中心室梗阻(MVO)是肥厚性心肌病(HCM)的一种罕见的表型亚变体。它发生在1-10%的患者中,由于其罕见,其临床进展和自然史尚未得到广泛研究。然而,现有资料显示其临床结果和预后较差。虽然菲律宾已经发表了关于肥厚性心肌病的报告,但在当地文献中没有关于这种特殊变异的数据。我们确定了3例肥厚性心肌病合并中心室梗阻。2例初步处理为缺血性心脏病,经心脏磁共振排除。其中一个病例最初因中风入院,然而,在二维超声检查中偶然发现HCM的存在。他还接受了心脏磁共振检查,这显示了他的疾病的全部程度。所有3例患者均发现因阻塞引起的压力缺血而发生根尖动脉瘤。肥厚性心肌病是最常见的遗传性心血管疾病,但其亚变体罕见且难以诊断。根尖动脉瘤在二维超声上也常被遗漏。然而,心脏磁共振提供了更多的疾病检测,更详细的范围和严重程度。它还可以提供有关心律失常和心源性猝死的预后信息。然而,需要更多的数据,以便在其应用上开辟新的研究途径。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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