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.

Yamaguchi syndrome or apical hypertrophic cardiomyopathy is a rare subtype of non-obstructive hypertrophic cardiomyopathy that is defined as the focused hypertrophy of the left ventricular apex. It is typically seen in Asian populations. Herein, we present a rare case of Yamaguchi syndrome seen in a Hispanic female.

Apical hypertrophic cardiomyopathy, also called Yamaguchi syndrome, is a rare variant of hypertrophic cardiomyopathy. Yamaguchi syndrome is characterized by hypertrophy almost confined to the apical region of the left ventricle rather than the left ventricular septum. A case of 65-year-old Saudi man presented to the ER with angina, and the ECG, echocardiogram, and nuclear study confirmed the diagnosis with Yamaguchi. Reporting this case serves to help physicians broaden their vision in approaching patients with symptoms mimicking acute coronary syndrome.

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该文报道1例青年男性患者因体检心电图异常就诊，进一步磁共振检查诊断为心尖肥厚型心肌病，在无心血管系统相关症状的情况下进展为中部梗阻并室壁瘤形成。本文通过心脏磁共振成像展示了心尖肥厚型心肌病进展形成室壁瘤的形态、功能及组织学变化，其中腺苷负荷状态下心肌灌注缺损揭示了冠状动脉血流储备减低、劳力性心肌缺血可能是室壁瘤形成原因之一。心脏磁共振在心尖肥厚型心肌病的随访复查中具有重要价值。.

BACKGROUND: Apical hypertrophic cardiomyopathy (ApHCM) is a phenotypic variant of hypertrophic cardiomyopathy. Endomyocardial fibrosis and endocardial calcification are especially rare in ApHCM.
METHODS: The main symptoms was chest tightness, palpitation, shortness of breath, and fatigue. Echocardiography and imaging examinations found apical hypertrophy along with endocardial calcification and endomyocardial fibrosis. Abnormal structural changes led to thrombosis and made the left ventricle a flat shape resembling an \"apple.\"
METHODS: The typical presentations, hypertrophic apex on echocardiography and an elevated N-terminal pro-brain natriuretic peptide level indicated the diagnosis of ApHCM and heart failure with preserved ejection fraction.
METHODS: Optimal medical therapy including the administration of ApHCM, heart failure and atrial fibrillation to improve symptoms and life quality.
RESULTS: Since discharge, the patient could perform normal daily activities and had no discomfort based on the optimal medical therapy.
CONCLUSIONS: We report a ApHCM patients with unusual presentations of endomyocardial fibrosis and apical calcification. This case highlights the importance of understanding the specific pathological changes of ApHCM for treatment and prognosis.

BACKGROUND: Apical hypertrophic cardiomyopathy (AHCM) is a subtype of hypertrophic cardiomyopathy. Due to its location, the thickening of the left ventricular apex can be missed on echocardiography. Giant negative T waves (GNTs) in left-sided chest leads are the hallmark electrocardiogram (ECG) change of AHCM.
METHODS: The first patient was a 68-year-old woman complaining of recurrent chest tightness persisting for more than 3 years. The second was a 59-year-old man complaining of spasmodic chest tightness persisting for more than 2 years. The third was a 55-year-old woman complaining of recurrent chest pain persisting for 4 mo. In all three cases, GNTs were observed several years prior to apical cardiac hypertrophy after other causes of T-wave inversion were ruled out.
CONCLUSIONS: Electrophysiological abnormalities of AHCM appear earlier than structural abnormalities, confirming the early predictive value of ECG for AHCM.

Apical hypertrophic cardiomyopathy is a rare variant of hypertrophic cardiomyopathy characterized by abnormal heart muscle thickening, specifically affecting the left ventricle\'s apex. Classically revealing both giant T-wave inversions in the precordial leads of an electrocardiogram and a spade-like configuration of the left ventricular cavity on ventriculograms, the diagnosis of the apical variant has evolved with cardiac magnetic resonance imaging. Despite being well known among East Asian populations, the diagnosis of apical hypertrophic cardiomyopathy is often underestimated and overlooked among American patients due to the non-specific nature of echocardiography. In this case report, we present the diagnosis of apical hypertrophic cardiomyopathy in a middle-aged African American male with chronic palpitations. The diagnosis was confirmed using cardiac magnetic resonance imaging, which revealed extensive myocardial fibrosis. Ultimately, the patient was treated with an implantable cardioverter-defibrillator. Our case aims to enhance the understanding and facilitate the recognition and management of apical hypertrophic cardiomyopathy, particularly among non-Asian individuals. Current challenges revolve around robust risk stratification strategies for patients at high risk for sudden cardiac death that require device therapy.

Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare phenotype of hypertrophic cardiomyopathy, which is characterized by focal thickening of the left ventricular (LV) apical myocardium, showing a spade-shaped shadow on the left ventricle. We present the case of a 59-year-old man who was found to have AHCM, is an asymptomatic orthotopic heart transplantation (HTx) patient. This rare and progressive case of LV apical hypertrophy emerged from the fourth year post surgery. We analyzed the etiology of this case and summarized the clinical manifestations and prognosis of AHCM following HTx by reviewing our case and the literature.

UNASSIGNED: Apical hypertrophic cardiomyopathy (ApHCM) is a rare form of hypertrophic cardiomyopathy which predominantly affects the apex of the left ventricle. The diagnosis can be challenging due to several factors, ranging from no typical clinical and electrocardiogram (EKG) findings to potential difficulties in executing and interpreting the echocardiographic examination.
UNASSIGNED: We report the case of an 84-year-old woman who came to our echo-lab to undergo a routine echocardiogram. She had a history of permanent atrial fibrillation, paced rhythm and previous episodes of heart failure (HF), allegedly explained by a diagnosis of hypertensive heart disease that had been confirmed many times over the previous 20 years. The clinical examination and the EKG were unremarkable. The echocardiographic images were poor quality. But a senior cardiologist, expert in imaging and echocardiography, noted the lack of delineation of the endocardial border of the left ventricular (LV) apex region. Contrast echocardiography was performed and severe apical hypertrophy discovered.
UNASSIGNED: ApHCM can be a challenging diagnosis. Contrast echocardiography must always be applied in cases of poor delineation of the LV apical endocardial border at baseline echocardiography. Timely detection and appropriate lifestyle intervention might slow the development of LV hypertrophy, and possibly minimize and delay heart failure (HF) related symptoms and arrhythmias. The prognosis remains relatively benign during long term follow-up.