Role of Imaging in the Detection of Reversible Cardiomyopathy

Park, Jae‐Hyeong; Kwon, Deborah; Starling, Randall C.; Marwick, Thomas H.

doi:10.4250/jcu.2013.21.2.45

Cited by 10 publications

(10 citation statements)

References 101 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It allows to differentiate FD from sarcomeric HCM, which usually results in LGE at the right ventricular (RV) junction points, and amyloidosis, which usually results in a global subendocardial pattern of LGE [51]. However, this LGE pattern is not exclusive of FD and may also be found in myocarditis, Chagas disease, and sarcoidosis [52]. A binary appearance of the LV endocardial border, known as the "binary sign," was correlated with a characteristic pattern of glycosphingolipid compartmentalization on histological examination and once thought to represent a hallmark feature of Fabry cardiomyopathy [42].…”

Section: Left Ventricular Storage Inflammation and Fibrosismentioning

confidence: 99%

Fabry Disease and the Heart: A Comprehensive Review

Azevedo

Cordeiro

Gago

et al. 2021

IJMS

View full text Add to dashboard Cite

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations of the GLA gene that result in a deficiency of the enzymatic activity of α-galactosidase A and consequent accumulation of glycosphingolipids in body fluids and lysosomes of the cells throughout the body. GB3 accumulation occurs in virtually all cardiac cells (cardiomyocytes, conduction system cells, fibroblasts, and endothelial and smooth muscle vascular cells), ultimately leading to ventricular hypertrophy and fibrosis, heart failure, valve disease, angina, dysrhythmias, cardiac conduction abnormalities, and sudden death. Despite available therapies and supportive treatment, cardiac involvement carries a major prognostic impact, representing the main cause of death in FD. In the last years, knowledge has substantially evolved on the pathophysiological mechanisms leading to cardiac damage, the natural history of cardiac manifestations, the late-onset phenotypes with predominant cardiac involvement, the early markers of cardiac damage, the role of multimodality cardiac imaging on the diagnosis, management and follow-up of Fabry patients, and the cardiac efficacy of available therapies. Herein, we provide a comprehensive and integrated review on the cardiac involvement of FD, at the pathophysiological, anatomopathological, laboratory, imaging, and clinical levels, as well as on the diagnosis and management of cardiac manifestations, their supportive treatment, and the cardiac efficacy of specific therapies, such as enzyme replacement therapy and migalastat.

show abstract

Section: Left Ventricular Storage Inflammation and Fibrosismentioning

confidence: 99%

Fabry Disease and the Heart: A Comprehensive Review

Azevedo

Cordeiro

Gago

et al. 2021

IJMS

View full text Add to dashboard Cite

show abstract

“…To improve cardiac function in CSS patients with DCMP, the early identification of cardiac involvement by multi-modality imaging and appropriate medical therapy would be of importance. 14) Considering the pathophysiologic mechanism of DCMP in patients with CSS, immunosuppressive therapy to control disease activity of CSS should be included, in addition to conventional medical therapy for heart failure including blocking agents for renin-angiotensin-aldosterone system and beta-blockade. The previous study have shown that high dose steroid and chemotherapy with cyclophosphamide are the most effective treatment of cardiac involvement of CSS, even though cyclophosphamide has a potential of cardiotoxicity.…”

Section: Discussionmentioning

confidence: 99%

Cardiac Involvement of Churg-Strauss Syndrome as a Reversible Cause of Dilated Cardiomyopathy

Jeong

Kim

Cho

et al. 2015

J Cardiovasc Ultrasound

View full text Add to dashboard Cite

A 31-year-old male who had been treated for Churg-Strauss syndrome (CSS) presented with sudden onset of dysarthria. Brain magnetic resonance imaging (MRI) showed acute multifocal bilateral cerebral infarctions suggesting embolic causes. Cardiac MRI showed dilated cardiomyopathy with severe biventricular dysfunction with intracardiac thrombi, and multiple high signal intensity spots in myocardium of the left ventricle with multifocal delayed enhancement suggesting multifocal myocarditis due to small vessel vasculitis associated with CSS. After anticoagulation therapy, treatments for heart failure, and immunosuppressive therapy including parenteral steroids and cyclophosphamide to control CSS, the symptoms and signs of heart failure and cardiac function of the patient were improved. Considering the pathophysiologic mechanism of cardiac involvement in CSS, immunosuppressive therapy to control the disease activity of CSS should be taken into account, besides usual management for heart failure.

show abstract

“…T1 mapping will show increased values; this is especially useful in patients with unremarkable LGE. Patients with edema have increased native T2 values compared with those in normal subjects [33,34] (Figs. 4 and 5).…”

Section: A B C D Ementioning

confidence: 99%

Clinical Application of T1 and T2 Mapping in Cardiac Magnetic Resonance Imaging for Nonischemic Cardiomyopathy: A Case-Based Review

Fundikira

Hong

Kim

et al. 2018

Cardiovasc Imaging Asia

View full text Add to dashboard Cite

Cardiac magnetic resonance imaging is instrumental in diagnosing various cardiovascular diseases. Recently introduced T1 mapping and T2 mapping sequences have enabled quantification of the T1 and T2 relaxation times of the myocardium and associated cardiovascular structures, which are intrinsic properties of tissues. These sequences have been increasingly used to diagnose cardiomyopathy based on changes in the T1 and T2 relaxation times and to objectively quantify the severity of cardiomyopathy. As reference values in T1 and T2 mapping sequences are influenced by specific techniques or magnetic field strength, they are limitations. However, parametric imaging with native T1, T2, and extracellular volume fraction (%) values yields a higher diagnostic accuracy than conventional MRI and is useful for diagnosis, treatment, and risk stratification of cardiomyopathy.

show abstract

Role of Imaging in the Detection of Reversible Cardiomyopathy

Cited by 10 publications

References 101 publications

Fabry Disease and the Heart: A Comprehensive Review

Fabry Disease and the Heart: A Comprehensive Review

Cardiac Involvement of Churg-Strauss Syndrome as a Reversible Cause of Dilated Cardiomyopathy

Clinical Application of T1 and T2 Mapping in Cardiac Magnetic Resonance Imaging for Nonischemic Cardiomyopathy: A Case-Based Review

Contact Info

Product

Resources

About