Hamza Izhan, Odigie-Okon Esosa, Xie Tianrong, Ahmad Masood
Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA.
Division of Cardiovascular Medicine, Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA.
Echocardiography. 2025 Jul;42(7):e70235. doi: 10.1111/echo.70235.
Apical Hypertrophic Cardiomyopathy (ApHCM) is a distinct phenotypic variant of hypertrophic cardiomyopathy, defined by localized thickening of the left ventricular (LV) apex ≥15 mm in end-diastole. It presents unique diagnostic and management challenges, with clinical presentations ranging from asymptomatic individuals to those experiencing angina, dyspnea, or arrhythmias. Transthoracic echocardiography remains the first-line imaging modality but is often limited by suboptimal apical visualization. Contrast-enhanced echocardiography is recommended to improve diagnostic accuracy, particularly for identifying apical hypertrophy, apical aneurysms, and thrombi. Echocardiography also plays a key role in assessing left ventricular outflow tract obstruction, detecting concomitant mitral valve abnormalities, and guiding periprocedural planning. Cardiac magnetic resonance imaging (CMR) provides superior spatial resolution and tissue characterization. It is the gold standard for evaluating apical wall thickness, detecting apical aneurysms, and quantifying myocardial fibrosis through late gadolinium enhancement. CMR-based markers such as LGE burden and aneurysm size are valuable for sudden cardiac death risk stratification. Additional imaging modalities, including cardiac computed tomography and nuclear perfusion imaging, are important adjuncts when CMR is contraindicated or when evaluation for coronary artery disease is necessary. This review underscores the central role of multimodality imaging in the diagnosis, risk assessment, and management of ApHCM. Future research should focus on refining risk prediction tools to improve individualized care and clinical outcomes in this unique cardiomyopathy subtype.
心尖肥厚型心肌病（ApHCM）是肥厚型心肌病的一种独特表型变异，定义为舒张末期左心室（LV）心尖局部增厚≥15毫米。它带来了独特的诊断和管理挑战，临床表现从无症状个体到出现心绞痛、呼吸困难或心律失常的患者不等。经胸超声心动图仍然是一线成像方式，但常常受到心尖部可视化不佳的限制。推荐使用对比增强超声心动图来提高诊断准确性，特别是用于识别心尖肥厚、心尖部动脉瘤和血栓。超声心动图在评估左心室流出道梗阻、检测合并的二尖瓣异常以及指导围手术期规划方面也起着关键作用。心脏磁共振成像（CMR）提供了卓越的空间分辨率和组织特征。它是评估心尖壁厚度、检测心尖部动脉瘤以及通过延迟钆增强定量心肌纤维化的金标准。基于CMR的标志物，如延迟钆增强负荷和动脉瘤大小，对于心脏性猝死风险分层很有价值。当CMR禁忌或需要评估冠状动脉疾病时，包括心脏计算机断层扫描和核灌注成像在内的其他成像方式是重要的辅助手段。本综述强调了多模态成像在ApHCM诊断、风险评估和管理中的核心作用。未来的研究应专注于完善风险预测工具，以改善这种独特心肌病亚型的个体化治疗和临床结局。
