Abstract
Endomyocardial diseases cardiomyopathy: A review and pooled analysis of pathophysiology, diagnosis and clinical management
Highlights
Cardiomyopathies (CM), as a non-coronary cause of heart failure (HF), was established in the 18th Century, and subsequently classified into four types: dilated (DCM), hypertrophic (HCM), restrictive (RCM) and arrhythmogenic cardiomyopathy [1,2,3]
endocardial fibroelastosis (EFE) patients were younger (7.5 days to 10.1 months) while endomyocardial fibrosis (EMF) and hypereosinophilic syndrome (HES) patients were relatively older (18±11 to 45±17), which mirrors the common age of presentation of the respective underlying endomyocardial disease
Other common features included peripheral emboli in 20.5% of the patients, posterior mitral leaflet in 29.3% of the patients, and intraventricular thrombus in 44.5%. These findings suggest that echocardiography is an important non-invasive diagnostic modality for evaluating cardiac manifestations of HES – thickening of postero-basal wall associated with impaired posterior mitral leaflet function resulting in mitral regurgitation and peripheral embolization and thrombus formation
Summary
Cardiomyopathies (CM), as a non-coronary cause of heart failure (HF), was established in the 18th Century, and subsequently classified into four types: dilated (DCM), hypertrophic (HCM), restrictive (RCM) and (more recently) arrhythmogenic cardiomyopathy [1,2,3]. The lack of consistency in the classification of CM due to endomyocardial diseases undermines comparative studies and subsequently a holistic understanding of its clinical course, pathophysiology and clinical management. Due to the rarity of the CM due to endomyocardial diseases, there is a clear lack of evidence on diagnosis and management yet the disease has an ominous prognosis if not detected early and prompt treatment initiated. This present systematic review and meta-analysis aggregates published evidence on endomyocardial diseases as a cause of CMs with a particular focus on pathophysiology, diagnosis and clinical management.
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