Aims A tailored chelation therapy guided by magnetic resonance imaging (MRI) is a strategy to improve the prognosis in iron-loaded patients, in many cases still hampered by limited MRI availability. In order to address this issue, the Myocardial Iron Overload in Thalassemia (MIOT) network was established in Italy and we aimed to describe the impact of 10-year activity of this network on cardiac burden in thalassemia major (TM). Methods and results Within the MIOT network, 1746 TM patients (911 females; mean age 31.2 ± 9.1 years) were consecutively enrolled and prospectively followed by 70 thalassemia and 10 MRI centres. Patients were scanned using a multiparametric approach for assessing myocardial iron overload (MIO), biventricular function, and myocardial fibrosis. At the last MRI scan, a significant increase in global heart T2* values and a significantly higher frequency of patients with no MIO (all segmental T2* ≥20 ms) were detected, with a concordant improvement in biventricular function, particularly in patients with baseline global heart T2* <20 ms. Forty-seven percentage of patients changed the chelation regimen based on MRI. The frequency of heart failure (HF) significantly decreased after baseline MRI from 3.5 to 0.8% (P < 0.0001). Forty-six patients died during the study, and HF accounted for 34.8% of deaths. Conclusion Over 10 years, continuous monitoring of cardiac iron and a tailored chelation therapy allowed MIO reduction, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related HF. A national networking for rare diseases therefore proved effective in improving the care and reducing cardiac outcomes of TM patients. Key Question Which was the impact on cardiac outcomes in thalassemia major by a national network among thalassemia and magnetic resonance imaging centres ensuring the continuous and standardized monitoring of the cardiac iron levels? Key Finding There was a reduction of myocardial iron overload (MIO) in almost 70% of patients, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related heart failure. Take Home Message A national clinical and imaging networking in rare diseases was effective in improving the care and in reducing the cardiac burden in thalassemia major patients.

In thalassemia major, pancreatic iron was demonstrated as a powerful predictor not only for the alterations of glucose metabolism but also for cardiac iron, fibrosis, and complications, supporting a profound link between pancreatic iron and heart disease. We determined for the first time the prevalence of pancreatic iron overload (IO) in thalassemia intermedia (TI) and systematically explored the link between pancreas T2* values and glucose metabolism and cardiac outcomes. We considered 221 beta-TI patients (53.2% females, 42.95 ± 13.74 years) consecutively enrolled in the Extension–Myocardial Iron Overload in Thalassemia project. Magnetic Resonance Imaging was used to quantify IO (T2* technique) and biventricular function and to detect replacement myocardial fibrosis. The glucose metabolism was assessed by the oral glucose tolerance test (OGTT). Pancreatic IO was more frequent in regularly transfused (N = 145) than in nontransfused patients (67.6% vs. 31.6%; p < 0.0001). In the regular transfused group, splenectomy and hepatitis C virus infection were both associated with high pancreatic siderosis. Patients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with altered OGTT. A pancreas T2* < 17.9 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for cardiac iron. Pancreas T2* values were not associated to biventricular function, replacement myocardial fibrosis, or cardiac complications. Our findings suggest that in the presence of pancreatic IO, it would be prudent to initiate or intensify iron chelation therapy to prospectively prevent both disturbances of glucose metabolism and cardiac iron accumulation.

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An electronic medical record system has been designed and developed with the aim of supportiq patient care in the departnzeizt of Cardiology. A clinical information system was implemented to integrate the different heterogeneous sources of patient information. All the clinically relevant patient data were timely gathered and stored into the clinical database. A viewer/editor of patient nzedical records was designed addressing friendliness, flexibility and communication. The basic function for consultation is the time-orietzted (weekly or daily) representation of patient parameters, care events and examinations. WEB technology has been applied to implement the medical record. The systern iy curreid)' under clinical evaluation. IntroductionIn spite of some decades of exploratory work and rnoney investments in research and development of computer systems in health care, patient records today are often paper-based [ I]. Implementation and deployment of electronic medical record (EMK) systems lag behind available technological solutions [ 2 ] . Of the several clinical information systems, which have become operational in recent years, only a few are capable of supporting the complete patient record and their deployment outside the development site is difficult. Basically the lack of standardization of both the structure and applications limits the diffusion of current EMR systems. Even though many data of clinical use are now available in electronic form, there still remains the need for health care personnel to report much of information in written form. This is the case of data gathered through patient consultation, referring to patient history and physical examinations, or physicians' and nurses' notes. Yet data capture from medical instrumentation is a crucial problem in clinical departments, given the lack of standard protocols for communication. Thus, timely and accurate transfer of patient information into the computer is a difficu!t step in the maintenance of an EMR. Interaction of clinicians with current computer systems is often laborintensive, thus provoking their rejection. Our effort, supported by a project of national Health Ministry for the management of health care delivery, addressed the above aspects to develop an EMK system for patient care in Cardiology. A computer-network infrastructure was realized to integrate the different clinical units, which concern patients accessing the health-care system. A variety of data, heterogeneous in nature (i.e. text, signals, and images), are gathered from each unit and stored into a relational database [3.1. Data are then retrieved from the database and presented to health-care personnel on clinical workstations. Friendliness and communication efficacy are addressed in the design of the EMR human-computer interface. Public-domain technologies have been preferred for developing viewededitors of multimedia documents and their transmission. World Wide Web (WEB) technology is used for the implementation of the EMR over the Internet without compromising confidential...

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