Cardiovascular magnetic resonance (CMR) in restrictive cardiomyopathies
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CARDIAC RADIOLOGY
Cardiovascular magnetic resonance (CMR) in restrictive cardiomyopathies Nicola Galea1,2 · Gesualdo Polizzi3 · Marco Gatti4 · Giulia Cundari2 · Michele Figuera3 · Riccardo Faletti4 Received: 9 June 2020 / Accepted: 3 September 2020 © The Author(s) 2020
Abstract The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. In this context, cardiac magnetic resonance (CMR) may play a crucial role in defining the diagnosis and guiding treatments, by offering a robust myocardial characterization based on the inherent magnetic properties of abnormal tissues, thus limiting the use of endomyocardial biopsy. In this review article, we explore the role of CMR in the assessment of a wide range of myocardial diseases causing restrictive patterns, from iron overload to cardiac amyloidosis, endomyocardial fibrosis or radiation-induced heart disease. Here, we emphasize the incremental value of novel relaxometric techniques such as T1 and T2 mapping, which may recognize different storage diseases based on the intrinsic magnetic properties of the accumulating metabolites, with or without the use of gadolinium-based contrast agents. We illustrate the importance of these CMR techniques and their great support when contrast media administration is contraindicated. Finally, we describe the useful role of cardiac computed tomography for diagnosis and management of restrictive cardiomyopathies when CMR is contraindicated. Keywords Restrictive cardiomyopathies · Infiltrative cardiomyopathies · Cardiovascular magnetic resonance · Cardiac imaging
Introduction Restrictive cardiomyopathy (RCM) is a myocardial disorder that is usually caused by increased myocardial stiffness which results in impaired ventricular filling. Until later stages of the disease, biventricular chamber size and systolic function are usually normal or almost normal. RCM include primary or idiopathic (a rare familial or sporadic genetic condition associated with the accumulation of desmin and
* Riccardo Faletti [email protected] 1
Department of Experimental Medicine, “Sapienza” University of Rome, Rome, Italy
2
Department of Radiological, Oncological and Pathological Sciences, “Sapienza” University of Rome, Rome, Italy
3
Unit of Radiodiagnostics II, University Hospital “Policlinico Vittorio Emanuele”, Catania, Italy
4
Department of Surgical Sciences, Radiology Unit, University of Turin, Turin, Italy
collagen type III) and secondary forms, which include infiltrative, non-infiltrative and storage disorders. RCMs are generally caused by processes causing abnormal deposition of proteins, glycogen and iron within the myocardium, leading to ventricular stiffness with diastolic dysfunction. The most common classification system [1] in adults divides restrictive cardiomyopathies on the basis of etiology
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