New perspectives of diagnosis and treatment for the different forms of cardiac amyloidosis

Thanks to research, new possibilities of treatment are opened for cardiac amyloidosis, a disease caused by an abnormal protein (amyloid), whose deposits in the heart tissue make it difficult for the heart to work properly. Cardiac amyloidosis is a clinical condition so far considered to be of rare occurrence which instead represents, to date, an increasingly frequent challenge for the cardiologist and internist. The new knowledge allows an early and non-invasive diagnosis, the ability to discriminate the different forms of the disease and to establish a targeted treatment, using new tools such as biomarkers, diagnostics by signal (electrocardiogram) and by image (echocardiogram, scintigraphy). and magnetic resonance), removing the often poor prognosis that, up to a recent past, accompanied cardiac amyloidosis in its various forms. The volume published by Pisa University Press for the “Sant’Anna Medical Reviews “entitled” Cardiac amyloidosis: how it is diagnosed, how it is treated “, which sees among the authors Michele Emdin and Claudio Passino, both professors of Cardiology at the Institute of Life Sciences of the Sant’Anna School of Advanced Studies in Pisa, and Giuseppe Vergaro , researcher affiliated with the Sant’Anna School of Advanced Studies, Cardiology managers of the Monasterio Foundation. The volume also includes the contribution of young researchers from the Cardiology group of the Sant’Anna School of Advanced Studies, the University of Pisa, the Monasterio Foundation and experts including Claudio Rapezzi, Federico Perfetto and Francesco Cappelli. After a tribute to Giampaolo Merlini and the School of Pavia, the volume of the “Sant’Anna Medical Reviews” series covers in an exhaustive way a range of topics of medical interest, from etiology (the study of the causes of the disease) to physiopathology to diagnostics (biomarkers and multimodal imaging) to arrive at the therapy of the different forms of this disease.As the authors point out, the different forms of cardiac amyloidosis are characterized by the accumulation in the interstitial space of amorphous material consisting of ‘amyloid fibrils’ which induces a clinical picture heart failure, it originates disturbances of the rhythm and conduction of the heart. Until recently, amyloidosis was considered a pathology with poor therapeutic prospects. In recent years, on the other hand, we have witnessed both the most frequent forms of cardiac amyloidosis, namely cardiac amyloidosis due to immunoglobulin light chains (Al) and transthyretin amyloidosis (Attr), in its ‘wild type’ and mutated forms. to an extraordinary increase in diagnostic skills and therapeutic possibilities that has disproved the concept of rare disease. “For these reasons, knowing and recognizing early cardiac amyloidosis in its various forms – remember Michele Emdin, Claudio Passino, Giuseppe Vergaro – is an imperative for the cardiologist, for the generalist doctor and for the many specialists, such as neurologists, internists, nephrologists, haematologists, involved in their clinical management “.