Clinical & Experimental Cardiology
Open Access

The Role of Echocardiography in Diagnosing Endocardial Fibroelastosis

Shen Jovel^{* *}Correspondence: Shen Jovel, Department of Cardiology, University of Lille, Lille, France, Email:

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Description

Endocardial Fibroelastosis (EFE) is a rare heart condition characterized by the thickening of the endocardium due to an abnormal increase in fibrous and elastic tissue. Primarily affecting infants and young children, EFE can lead to significant morbidity and mortality if not diagnosed and treated early. Echocardiography, a non-invasive imaging technique, plays a key role in the diagnosis and management of EFE. This article explains the importance of echocardiography in identifying EFE, its diagnostic features, and its advantages over other diagnostic modalities.

EFE is marked by the thickening of the heart's endocardial layer, often leading to restrictive cardiomyopathy. The condition can be primary (idiopathic) or secondary, associated with various congenital heart defects, infections, or metabolic disorders. Symptoms of EFE include heart failure, respiratory distress, poor growth, and fatigue. Early and accurate diagnosis is important for effective management and improved outcomes. Echocardiography is the gold standard for diagnosing EFE. It provides detailed images of the heart’s structure and function, allowing clinicians to assess the thickness and elasticity of the endocardium. The following sections detail how echocardiography aids in diagnosing EFE. Increased echogenicity of the endocardium, particularly in the left ventricle, is a hallmark of EFE. This thickening can be uniform or patchy and is best visualized using two-dimensional echocardiography. EFE often leads to dilation of the left ventricle due to impaired contractility. This dilation can be quantified using M-mode echocardiography and two-dimensional imaging. Echocardiography can identify secondary effects of EFE on the heart valves, such as mitral or aortic valve regurgitation due to altered ventricular geometry. By measuring the ejection fraction, echocardiography helps assess the systolic function of the heart. EFE typically results in reduced ejection fraction due to impaired ventricular contractility. Doppler echocardiography can evaluate diastolic function, revealing restrictive filling patterns indicative of EFE. These patterns include decreased early diastolic filling and increased atrial contraction.

Tissue Doppler Imaging (TDI) assesses myocardial velocities, providing insights into both systolic and diastolic function. In EFE, TDI often shows reduced myocardial velocities, indicating impaired myocardial relaxation. Speckle Tracking Echocardiography (STE) analyzes myocardial deformation, offering a detailed evaluation of ventricular strain. Reduced strain patterns are common in EFE, reflecting compromised myocardial contractility. This technique offers comprehensive visualization of the endocardium, enhancing the detection of focal or diffuse thickening. Unlike cardiac catheterization or biopsy, echocardiography is non-invasive, reducing the risk of complications. Echocardiography provides real-time images, allowing for immediate assessment of the heart’s structure and function. Echocardiography is widely available and can be performed at the bedside, making it accessible for critically ill patients. Compared to Magnetic Resonance Imaging (MRI), echocardiography is more cost-effective, making it a practical choice for initial diagnosis and follow-up. While echocardio- graphy is invaluable in diagnosing EFE, it has limitations. Image quality can be compromised in patients with poor acoustic windows, and the technique may be less effective in detecting early or mild forms of EFE. In such cases, complementary imaging modalities like MRI can provide additional information. MRI offers superior soft tissue contrast and can accurately quantify ventricular volumes and fibrosis extent. It is particularly useful in ambiguous cases where echocardiographic findings are inconclusive. Though invasive, biopsy remains the definitive method for diagnosing EFE, particularly in complex cases where imaging findings are equivocal.

Conclusion

Echocardiography is a cornerstone in the diagnosis of endocardial fibroelastosis, providing critical information on the structural and functional abnormalities associated with the condition. Its noninvasive nature, real-time imaging capabilities, and wide availability make it an indispensable tool for clinicians. While echocardiography has some limitations, it remains the primary diagnostic modality for EFE, supplemented by advanced techniques and complementary imaging modalities when necessary. Early and accurate diagnosis through echocardiography is essential for effective management and improved outcomes in patients with EFE.