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Kilner P.J.,CMR Unit
Current Cardiovascular Imaging Reports | Year: 2010

Cardiovascular magnetic resonance (CMR) has important contributions to make to the assessment of heart valve disease. These can be complementary to routine echocardiographic assessment, for example in the quantification of valve regurgitation and clarification of the nature and level(s) of right or left ventricular outflow tract obstruction. In ischemic mitral regurgitation, CMR allows the assessment of myocardial scarring and viability as well as the nature of valve dysfunction. CMR provides a noninvasive alternative to echocardiography in patients with inconsistent findings or limited acoustic access. For studies of multidirectional flow, CMR can measure all three directional components of velocity in voxels distributed in three dimensions and through the phases of the cycle. More clinically applicable, however, are volumetric flow measurements, forward or regurgitant, through planes transecting one or both great arteries. These derived measurements are prone to errors caused by slight background phase offsets, which may require appropriate correction. © 2010 Springer Science+Business Media, LLC.

Roghi A.,CMR Unit
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance | Year: 2011

The clinical presentation of pheochromocytoma is variable and many biochemical and imaging methods have been suggested to improve the diagnostic accuracy of what has been termed "the great masquerader". This case-report is of a middle-aged woman with a non-specific clinical presentation suggesting acute coronary syndrome or subacute myocarditis. Cardiovascular magnetic resonance (CMR) at presentation showed myocardial edema and intramyocardial late gadolinium enhancement (LGE). An adrenal mass was seen, which was confirmed as pheochromocytoma and surgically removed. Our case shows evidence for acute adrenergic myocarditis, with resolution of both the edema and the LGE after surgical excision.

Cassinerio E.,University of Milan | Orofino N.,University of Milan | Roghi A.,CMR Unit | Duca L.,University of Milan | And 4 more authors.
Blood Cells, Molecules, and Diseases | Year: 2014

The availability of three iron chelators improved the scenario of chelation therapy for transfusion-dependent thalassemia (TDT) patients, allowing tailoring of drugs according to the goals expected for each patient. The use of Deferiprone/Deferoxamine (DFP/DFO) combined in different schemes has been reported since many years. Only recently data from combination of Deferasirox/Deferoxamine (DFX/DFO) have been reported showing that it can be safe and efficacious to remove iron overload, particularly in patients who do not respond adequately to a single chelating agent. We investigated the efficacy, tolerability and safety of combined DFX/DFO in thalassemia major patients. Ten TDT patients have started DFX/DFO for different reasons: 1) lack of efficacy in removing liver/cardiac iron with monotherapy; 2) agranulocytosis on DFP; and 3) adverse events with elevated doses of monotherapies. The study design included: cardiac and hepatic T2* magnetic resonance (CMR), transient elastography evaluation (Fibroscan), biochemical evaluation, and audiometric and ocular examinations. The drugs' starting doses were: DFO 32. ±. 4. mg/kg/day for 3-4. days a week and DFX 20. ±. 2. mg/kg/day. Seven patients completed the one-year follow-up period. At baseline the mean pre-transfusional Hb level was 9.4. ±. 0.4. g/dl, the mean iron intake was 0.40. ±. 0.10. mg/kg/day, the median ferritin level was 2254. ng/ml (range 644-17,681. ng/ml). Data available at 1. year showed no alteration of renal/hepatic function and no adverse events. A marked reduction in LIC (6.54 vs 11.44. mg/g dw at baseline) and in median ferritin (1346 vs 2254. ng/ml at baseline) was achieved. A concomitant reduction of non-transferrin-bound iron (NTBI) at six months was observed (2.1. ±. 1.0 vs 1.7. ±. 1.2. μM). An improvement in cardiac T2* values was detected (26.34. ±. 15.85 vs 19.85. ±. 12.06 at baseline). At 1. year an increased dose of DFX was administered (27. ±. 6. mg/kg/day vs 20. ±. 2. mg/kg/day at baseline, p. = 0.01) with a stable dose of DFO (32. ±. 4. mg/kg/day). Combined or alternated DFX/DFO can be considered when monotherapy is not able to remove the iron overload or in the presence of adverse events. © 2014 Elsevier Inc.

Kilner P.J.,CMR Unit | Geva T.,Havard Medical School | Kaemmerer H.,Deutsches Herzzentrum | Trindade P.T.,University of Zurich | And 2 more authors.
European Heart Journal | Year: 2010

This paper aims to provide information and explanations regarding the clinically relevant options, strengths, and limitations of cardiovascular magnetic resonance (CMR) in relation to adults with congenital heart disease (CHD). Cardiovascular magnetic resonance can provide assessments of anatomical connections, biventricular function, myocardial viability, measurements of flow, angiography, and more, without ionizing radiation. It should be regarded as a necessary facility in a centre specializing in the care of adults with CHD. Also, those using CMR to investigate acquired heart disease should be able to recognize and evaluate previously unsuspected CHD such as septal defects, anomalously connected pulmonary veins, or double-chambered right ventricle. To realize its full potential and to avoid pitfalls, however, CMR of CHD requires training and experience. Appropriate pathophysiological understanding is needed to evaluate cardiovascular function after surgery for tetralogy of Fallot, transposition of the great arteries, and after Fontan operations. For these and other complex CHD, CMR should be undertaken by specialists committed to long-term collaboration with the clinicians and surgeons managing the patients. We provide a table of CMR acquisition protocols in relation to CHD categories as a guide towards appropriate use of this uniquely versatile imaging modality.

Cassinerio E.,Foundation Medicine | Roghi A.,CMR Unit | Orofino N.,Foundation Medicine | Pedrotti P.,CMR Unit | And 6 more authors.
Annals of Hematology | Year: 2015

Deferasirox (DFX) is an oral iron chelator with established efficacy and safety. We evaluated by T2* cardiovascular magnetic resonance (CMR) the efficacy of DFX in preventing and removing cardiac and liver iron load and cardiac volume changes, along 5 years in adult thalassemia major (TM) patients. Twenty-three TM patients (9 males/14 women, mean age 36 ± 4 years) were included in this study. Repeated CMR was performed to assess myocardial and liver iron load (baseline t0, after 2.5 years t1, after 5 years t2). Myocardial T2* values changed progressively and increased significantly between t0 and t2 (t0: 27.15 ± 9.58 vs t2: 36.64 ± 6.68, p = 0.0001). At baseline evaluation, a cardiac T2* value <20 ms was detected in six patients (26 %): they showed an improvement of cardiac T2* values between t0 and t1, with normal T2* levels reached in all patients at t2. In the overall population, a significant reduction of both end-diastolic and end-systolic left ventricular volumes (EDV, ESV) were detected between t0 and t2 (EDV, t0: 132 ± 31 ml vs t2: 124 ± 22 ml, p = 0.033; ESV, t0: 48 ± 14 ml vs t2: 41 ± 10 ml, p = 0.0007). A significant reduction in liver iron concentration (LIC) was detected at t1 (5.36 ± 3.58 mg/g dw at baseline vs 3.35 ± 2.68 mg/g dw at t1, p = 0.004). In patients with cardiac iron overload at baseline (n.6), mean cardiac T2* values doubled at t2, and mean LIC value is reduced of 29 %. After 5 years of treatment, DFX continually and significantly reduced myocardial and liver iron overload, and it prevented further iron deposition. © 2015, Springer-Verlag Berlin Heidelberg.

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