We systematically explored the link of pancreatic iron with glucose metabolism and with cardiac complications in a cohort of 1,079 patients with thalassemia major (TM) enrolled in the Extension-Myocardial Iron Overload in Thalassemia (E-MIOT) project. RESEARCH DESIGN AND METHODSMRI was used to quantify iron overload (T2* technique) and cardiac function (cine images) and to detect macroscopic myocardial fibrosis (late gadolinium enhancement technique). Glucose metabolism was assessed by the oral glucose tolerance test (OGTT). RESULTSPatients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with impaired fasting glucose, impaired glucose tolerance, and diabetes. A pancreas T2* <13.07 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for disturbances of glucose metabolism and for cardiac iron. Patients with myocardial fibrosis showed significantly lower pancreas T2* values. Patients with cardiac complications had significantly lower pancreas T2* values. No patient with arrhythmias/heart failure had a normal global pancreas T2*. CONCLUSIONSPancreatic iron is a powerful predictor not only for glucose metabolism but also for cardiac iron and complications, supporting the close link between pancreatic iron and heart disease and the need to intensify iron chelation therapy to prevent both alterations of glucose metabolism and cardiac iron accumulation.Thalassemia major (TM) is a hereditary anemia characterized by ineffective erythropoiesis and hemolysis, requiring regular red blood cell transfusions to sustain life. A major drawback of this treatment is iron overload, which can cause organ dysfunction (1). The introduction of chelation therapy has improved the survival of TM patients, but cardiac complications remain the main cause of mortality, while endocrinopathies are the most frequent morbidities. Diabetes is the third-most-common endocrine complication (2). In a large cohort of well-treated and well-chelated TM patients, the

Implementation of a multidisciplinary thoracic malignancy conference increased the 1-year survival rate of patients who underwent a surgical resection for NSCLC.

No abstract

Summary We prospectively assessed the efficacy of deferasirox versus deferiprone or desferrioxamine as monotherapy in thalassaemia major (TM) patients by magnetic resonance imaging (MRI). We selected the patients enrolled in the Myocardial Iron Overload in Thalassaemia network who received only one chelator between two MRIs (deferasirox = 235, deferiprone = 142, desferrioxamine = 162). Iron overload was measured by T2* technique and biventricular function by cine images. Among the patients with baseline myocardial iron, in all three groups there was a significant improvement in global heart T2* values. The deferiprone and desferrioxamine groups showed a significant improvement in left ventricular ejection fraction (LVEF). Only the deferiprone group showed a significant improvement in right ventricular ejection fraction (RVEF). The improvement in global heart T2* was significantly lower in the deferasirox versus the deferiprone group. The improvement in the LVEF was significantly higher in the deferiprone and desferrioxamine groups than in the deferasirox group and the improvement in the RVEF was significantly higher in the deferiprone than in deferasirox group. Among the patients with baseline hepatic iron, the changes in hepatic iron were comparable in deferasirox versus the other groups. Deferasirox monotherapy was less effective than deferiprone in improving myocardial siderosis and biventricular function and less effective than desferrioxamine in improving the LVEF.

ST-segment elevation MI (STEMI) is a rare presentation in patients with coronary artery anomalies. In these patients, the identification of the culprit lesion and its treatment may be difficult, particularly in the emergency setting of primary percutaneous coronary intervention (PCI). From January 2008 to April 2011, 1015 STEMI patients received coronary artery angiography and primary PCI in our centre. Of these, 5 (0.4%) patients showed a coronary artery anomaly. In this paper we reported two rare cases: i) the first is a single coronary artery originating from right sinus of Valsalva; ii) the second is a separate origin of 3 coronary arteries originating from the right sinus of Valsalva. In conclusion, coronary artery anomalies presenting with STEMI are really uncommon, but often are a challenge. The integration between traditional coronary artery angiography and multidetector computerized tomography is crucial to optimize the interventional and medical management of these patients.

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* &lt;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 &lt; 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 patients with carotid atherosclerosis, ongoing CE-MRA with a neurovascular coil for the simultaneous detection of unstable plaques is feasible. Our MR protocol accurately identifies the major features of vulnerable plaque.

Background: Cardiac MRI plays a critical role in the management of thalassemic patients. No accurate biventricular reference values are available. Purpose: To establish the ranges for normal left ventricular (LV) and right ventricular (RV) volumes and ejection fraction (EF) and LV mass normalized to body surface area (BSA), age, and gender in a large cohort of well-treated betathalassemia major (β-TM) patients without heart damage using a multiparametric MRI. Study Type: Retrospective/cohort study. Population: In all, 251 β-TM patients with no known risk factors or cardiac disease, normal electrocardiogram, no macroscopic myocardial fibrosis, and all cardiac segments with T 2 * ≥20 msec, and 246 healthy subjects. Field Strength/Sequence: 1.5T/cine steady-state free precession (SSFP), gradient-echo T 2 *, late gadolinium enhancement (LGE) images. Assessment: Biventricular end-diastolic volume, end-systolic volume, stroke volume, and LV mass were normalized to BSA (EDVI, ESVI, SVI). Statistical Tests: Comparisons between the two groups was performed with two-samples t-test or Wilcoxon's signed rank test. For more than two groups, one-way analysis of variance (ANOVA) or a Kruskal-Wallis test were applied. Results: Compared to controls, males with β-TM showed significantlt higher LVEDVI in all the age groups, while for the other volumes the difference was significant only within one or more age groups. In females the volumes were comparable between β-TM patients and healthy subjects in all the age groups. In the male β-TM population we found a significant effect of age on LVEDVI