Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co‐morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.

中文翻译：

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使用多模态成像评估心尖肥厚型心肌病的心肌内钙化：病例系列

心尖肥厚型心肌病 (ApHCM) 是 HCM 的一种变体，经常影响中年男性。其特征是心尖闭塞和持续舒张期收缩，常常导致微血管缺血。我们报告了 5 例 ApHCM 病例，超声心动图显示心肌内钙化证据。在心脏磁成像 (MRI) 上，早期钆增强 (EGE) 序列的低信号成分与钙相容，而深层，晚期钆增强 (LGE) 序列的高信号与纤维化有关，提示心内膜心肌纤维化 (EMF) ） 诊断。 EMF 的病理标志是心内膜和心肌疤痕，并演变成营养不良性钙化。仅在少数 ApHCM 患者中发现。我们的系列是迄今为止描述的最大的系列。分析患者的病史，所有患者都存在明显的共存炎症触发因素，因此他们的合并症可能是小血管疾病的进一步原因，在肥大导致缺血性微血管应激的背景下，导致纤维化和营养不良性钙化。该系列可以证明根尖纤维化/钙化与肥大和炎症触发引起的微血管缺血之间的关系。