Echocardiography in Infiltrative Cardiomyopathy

Abstract

Left ventricular (LV) wall thickening can occur due to both physiological and pathological processes. Some LV wall thickening is caused by infiltrative cardiac deposition diseases-rare disorders from both inherited and acquired conditions, with varying systemic manifestations. They portend a poor prognosis and are generally not reversible except in rare circumstances when early diagnosis and treatment may alter the outcome (e.g., Fabry disease). Cardiac involvement is variable and depends on the degree of infiltration and type of infiltrate. These changes often lead to the development of abnormalities in both the relaxation and contractile function of the heart ultimately resulting in heart failure. Echocardiography is generally the first investigation of choice as it is easily available and gives valuable information about the thickness of the ventricular walls as well as systolic and diastolic function. It is also able to identify unique, characteristic features of the disease as well as detecting any haemodynamic sequelae. This review looks at the role of echocardiography in the diagnosis and prognosis of infiltrative cardiac deposition diseases.

Echocardiography: Navigating Complexities to Provide Many Useful Applications in Contemporary Clinical Cardiology

“…Alongside the technological advances, we have seen increasing applications of echocardiography in various clinical settings. In their review, Perry and Selvanayagam demonstrate the usefulness of echocardiography in patients with abnormal thickening of the LV walls due to cardiac involvement in infiltrative diseases such as amyloidosis, sarcoidosis and the potentially reversible Anderson-Fabry disease [10]. Advanced echocardiographic techniques may be especially helpful, both for the diagnosis and differentiation of the various aetiologies.…”

“…Amyloid deposition in the right ventricle (RV) occurs later than in the LV, and is indicative of a worse prognosis. In cardiac sarcoidosis, although Perry and Selvanayagam say more research is required, speckle tracking strain shows promise in detecting the condition, and particularly in patients who do not have access to, or who have a contraindication to CMR or position emission tomography [10]. In Anderson-Fabry disease, tissue Doppler imaging (TDI) may indicate cardiomyopathy progression and assist with the decision for early intervention with enzyme replacement therapy.…”

Echocardiography in patients with heart failure: recent advances and future perspectives

“…Besides the ability to reflect global myocardial performance, deformation parameters can reveal segmental differences in cardiac mechanics. These measurements provide incremental value in diagnosing diseases characterized by the regional heterogeneity of LV function like amyloidosis (relative preservation of apical strain) or Anderson-Fabry disease (functional impairment mainly in the mid/basal postero-lateral segments) [12].…”

A sonographer's guide to the clinical utility of left ventricular speckle tracking strain

“…16 This may be due to a compensative mechanism of increased radial contractility to counteract the reduced longitudinal contractility in early LV dysfunction occurring in the absence of ischemia or LV hypertrophy. 17…”