Abstract
The heart may be involved in a number of systemic syndromes. The pericardium, myocardium, heart valves, and coronary arteries may be involved either singly or in various combinations. In most cases the cardiac manifestations are not the dominant feature, but in some it is the primary determinant of symptoms and survival. Both the early identification of cardiac involvement and the etiology underneath is of paramount importance, as some causes require specific treatment and may be correctable. In this respect non-invasive imaging plays a central role especially in the context of rare cardiac disease, where specific imaging features can help to make the appropriate diagnosis on a substantial proportion of them, enabling the physician to choose the best management strategy tailored to the disease. Whereas echocardiography is the firstline investigation for detecting a cardiac involvement in systemic disease, cardiovascular magnetic resonance (CMR) provides additional incremental data allowing in addition to a detailed examination of cardiac structure and function also the tissue characterization. The aim of this review is therefore to delineate the role of CMR in detecting cardiac involvement in patients with rare systemic diseases and delineate the specific imaging features of the different etiologies.
Highlights
Gives very little information about tissue char- ©Copyright A
Whereas echocardiography is the firstline investigation for detecting a cardiac o involvement in systemic disease, cardiovascu-c lar magnetic resonance (CMR) provides additional incremental data allowing in addition to n a detailed examination of cardiac structure o and function the tissue characterization
Unique to this imaging modality, that has revolutionized the role of CMR in the evaluation of cardiac disease, is the tissue characterization
Summary
Patchy transmural pattern with characteristic zebra-stripe appearance sparing the midwall of the interventricular septum Anteroseptal and inferolateral mid-myocardial or epicardium involvement in an unpredictable distribution Midwall LGE distribution with a linear pattern, sparing the subendocardium and epicardium in basal and mid-cavity segments of the LV Predilection for basal inferolateral LV segments sparing subendocardium Exclusive involvement of endomyocardium Extensive multifocal LGE in mid myocardium and other areas Extensive mid-myocardial LGE. The usefulness of CMR in SSc has been m recently underlined, focusing on LGE abnormalities.[26] Late enhancement was characterism tically midwall, with a linear pattern, sparing o the subendocardium and epicardium (Table c 1). Smedema JP, Snoep G, van Kroonenburgh ly Eosinophilic diseases n Cardiac hyper-eosinophilia (e.g., malignant, o Loefflers; Churgg-Strauss) can cause endomyocardial fibrosis, valve disease and papillary e muscle dysfunction, diastolic dysfunction, s intracardiac thrombus formation, and heart u failure These features are highly characterisl tic and well diagnosed by CMR: T2-weighted sequences may monitor disease activity by the ia identification of edematous areas associated c with inflammation, early gadolinium techr nique can detect apical thrombus with high e diagnostic accuracy and late gadolinium imaging allows the detection of endomyocardial m fibrosis (Figure 1). The exclusive involvement of endomyocardium on LGE-CMR in conjuncm tion with normal myocardial function, apical o obliteration, and thrombus formation are typic cal findings in these patients[36,37,38] highlighting - the diagnostic role of CMR, a comprehensive n tool for the early diagnosis of this rare but No severe disease
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