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.