Histologic Sequelae of Apical Hypertrophic Cardiomyopathy: Dystrophic Calcification

Saba, Shahryar G.; Makaryus, John N.; Rahmani, Navid; Jadonath, Ram

doi:10.1177/1179546817710934

Cited by 3 publications

(4 citation statements)

References 6 publications

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“…These hemodynamic effects may lead to a pathophysiological cascade of infarction, fibrosis, and dystrophic calcification. [ 5 ] EMF and myocardial calcification in this case further limited the wall motion and ventricular diastolic filling, ultimately developing into severe heart failure. Changes in the structure of the cardiac chamber, such as hypertrophic calcified apex and ventricular septal aneurysm, would result in stasis of blood flow, which predispose to thrombus formation.…”

Section: Discussionmentioning

confidence: 92%

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Endomyocardial fibrosis and apical calcification: A case report with unusual presentations of apical hypertrophic cardiomyopathy

Gao,

Zhang,

Xie

et al. 2023

Medicine

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Rationale: Apical hypertrophic cardiomyopathy (ApHCM) is a phenotypic variant of hypertrophic cardiomyopathy. Endomyocardial fibrosis and endocardial calcification are especially rare in ApHCM. Patient concerns: 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.” Diagnoses: 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. Interventions: Optimal medical therapy including the administration of ApHCM, heart failure and atrial fibrillation to improve symptoms and life quality. Outcomes: Since discharge, the patient could perform normal daily activities and had no discomfort based on the optimal medical therapy. Lessons: 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.

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Section: Discussionmentioning

confidence: 92%

“…Previous reports [ 5 , 6 ] had described apical calcification in ApHCM. Myocardial calcification can be caused by several etiologies.…”

Section: Discussionmentioning

confidence: 94%

Endomyocardial fibrosis and apical calcification: A case report with unusual presentations of apical hypertrophic cardiomyopathy

Gao,

Zhang,

Xie

et al. 2023

Medicine

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“…However, in clinical practice, it is evident as only a minority of ApHCM patients develops EMF and calcifications. Actually, in literature only, seven cases of EMF in ApHCM patients are described in six different papers, 7 , 8 , 9 , 10 , 11 , 12 so our clinical series is the largest one.…”

Section: Discussionmentioning

confidence: 99%

Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series

Radano,

Mabritto,

Luceri

et al. 2024

ESC Heart Failure

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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.

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“…AHCM is a morphologic variant of hypertrophic cardiomyopathy. [1,4] Recently, apical EMF and calcification have been reported, [5–9] but their precise cause and effects remain unclear. The patients in this study had LV enlargement and EF >55%.…”

Section: Discussionmentioning

confidence: 99%

Apical hypertrophic cardiomyopathy with apical endomyocardial fibrosis and calcification

et al. 2019

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Rationale: Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy which affects predominantly the apex of the left ventricle. Generally, left ventricular enlargement is not present in AHCM; additionally, endomyocardial fibrosis, and calcification are also rare. Patient concerns: A 61-year-old female (Case 1) and a 60-year-old female (Case 2) both presented with the symptoms of atypical chest pain, dyspnoea, exercise intolerance, palpitations. Diagnosis: Magnetic resonance and single-photon emission computed tomography (SPECT) revealed apical hypertrophic cardiomyopathy. Furthermore, 2D-transthoracic echocardiogram showed left atrium and ventricular enlargement, as well as endomyocardial fibrosis and calcification. Based on these findings, the patients were diagnosed with AHCM. Interventions: Both the patients were treated with ACEI, metoprolol, and aspirin. Additionally, both these patient underwent genetic test. Outcomes: The results of the genetic test of the 2 cases for hypertrophic cardiomyopathy (HCM) were negative. However, the gene mutation for dilated cardiomyopathy (TMPO) was detected in one of the cases. No change in condition during follow-up. Lessons: In past reports, Apical hypertrophic cardiomyopathy has been shown to have a benign prognosis. But in this case report, the imaging studies of the 2 patients suggest a poor prognosis. Furthermore, diagnosing cardiomyopathy should require multimodality imaging examinations to rule out differential diagnoses.

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Histologic Sequelae of Apical Hypertrophic Cardiomyopathy: Dystrophic Calcification

Cited by 3 publications

References 6 publications

Endomyocardial fibrosis and apical calcification: A case report with unusual presentations of apical hypertrophic cardiomyopathy

Endomyocardial fibrosis and apical calcification: A case report with unusual presentations of apical hypertrophic cardiomyopathy

Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series

Apical hypertrophic cardiomyopathy with apical endomyocardial fibrosis and calcification

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