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Left ventricle remodeling in patients with β-thalassemia major. An emerging differential diagnosis with left ventricle noncompaction disease

      Highlights

      • Differentiation of remodeled left ventricle myocardium of β-thalassemia major from left ventricle (LV) non-compaction disease using cardiac magnetic resonance (CMR) imaging is still a challenging task.
      • Among the most used CMR criteria, those based on the percentage of non-compacted LV mass are better than the ratio of diastolic non-compacted to compacted myocardium at a segmental level.

      Abstract

      To differentiate left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to LV remodeling in β-thalassemia major (β-TM) patients, cardiac magnetic resonance (CMR) images of 38 β-TM patients and 10 LVNC patients were compared using 3 diagnostic criteria: ratio of diastolic segmental non-compacted to compacted myocardium (NC/C ratio) >2.5, percentage of non-compacted LV mass (NC-LVM%) >20% and >25% of global LV mass. Specificity of NC/C ratio of >2.5 was the lowest (58%) and of NC-LVM% of >25% the highest (93%). A NC-LVM% >20% showed sensitivity 100% and specificity 87%. Disease differentiation depends on the selected CMR criterion and is better with NC-LVM%.

      Keywords

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      References

        • Pennell D.J.
        • Udelson J.E.
        • Arai A.E.
        • Bozkurt B.
        • Cohen A.R.
        • Galanello R.
        • et al.
        Cardiovascular function and treatment in beta-thalassemia major: a consensus statement from the American Heart Association.
        Circulation. 2013; 128: 281-308
        • Marsella M.
        • Borgna-Pignatti C.
        • Meloni A.
        • Caldarelli V.
        • Dell'Amico M.C.
        • Spasiano A.
        • et al.
        Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a T2* magnetic resonance imaging study.
        Haematologica. 2011; 96: 515-520
        • Luckie M.
        • Irwin B.
        • Nair S.
        • Greenwood J.
        • Khattar R.
        Left ventricular non-compaction in identical twins with thalassaemia and cardiac iron overload.
        Eur J Echocardiogr. 2009; 10: 509-512
        • Piga A.
        • Longo F.
        • Musallam K.M.
        • Veltri A.
        • Ferroni F.
        • Chiribiri A.
        • et al.
        Left ventricular noncompaction in patients with β-thalassemia: uncovering a previously unrecognized abnormality.
        Am J Hematol. 2012; 87: 1079-1083
        • Oechslin E.N.
        • Attenhofer Jost C.H.
        • Rojas J.R.
        • Kaufmann P.A.
        • Jenni R.
        Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis.
        J Am Coll Cardiol. 2000; 36: 493-500
        • Stollberger C.
        • Finsterer J.
        • Blazek G.
        Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders.
        Am J Cardiol. 2002; 90: 899-902
        • Ritter M.
        • Oechslin E.
        • Sutsch G.
        • Attenhofer C.
        • Schneider J.
        • Jenni R.
        Isolated noncompaction of the myocardium in adults.
        Mayo Clin Proc. 1997; 72: 26-31
        • Petersen S.E.
        • Selvanayagam J.B.
        • Wiesmann F.
        • Robson M.D.
        • Francis J.M.
        • Anderson R.H.
        • et al.
        Left ventricular non-compaction. Insight from cardiovascular magnetic resonance imaging.
        J Am Coll Cardiol. 2005; 46: 101-105
        • Jacquier A.
        • Thuny F.
        • Jop B.
        • Giorgi R.
        • Cohen F.
        • Gaubert J.Y.
        • et al.
        Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular noncompaction.
        Eur Heart J. 2010; 31: 1098-1104
        • Grothoff M.
        • Pachowsky M.
        • Hoffmann J.
        • Posch M.
        • Klaassen S.
        • Lehmkuhl L.
        • et al.
        Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies.
        Eur Radiol. 2012; 22: 2699-2709
        • Meloni A.
        • Ramazzotti A.
        • Positano V.
        • Salvatori C.
        • Mangione M.
        • Marcheschi P.
        • et al.
        Evaluation of a web-based network for reproducible T2* MRI assessment of iron overload in thalassemia.
        Int J Med Inform. 2009; 78: 503-512
        • Jenni R.
        • Oechslin E.
        • Schneider J.
        • Attenhofer Jost C.H.
        • Kaufmann P.A.
        Echocardiographic and pathoanatomical characteristics of isolated LV non-compaction: a step towards classification as a distinct cardiomyopathy.
        Heart. 2001; 86: 666-671
        • Pepe A.
        • Positano V.
        • Santarelli M.F.
        • Sorrentino F.
        • Cracolici E.
        • De Marchi D.
        • et al.
        Multislice multiecho T2* cardiovascular magnetic resonance for detection of the heterogeneous distribution of myocardial iron overload.
        J Magn Reson Imaging. 2006; 23: 662-668
        • Heiberg E.
        • Sjogren S.
        • Ugander M.
        • Carlsson M.
        • Engblom H.
        • Arheden H.
        Design and validation of segment-freely available software for cardiovascular image analysis.
        BMC Med Imaging. 2012; 10: 1
        • Maceira A.M.
        • Prasad S.K.
        • Khan M.
        • Pennell D.J.
        Normalized left ventricular systolic and diastolic function by steady state free precession cardiovascular magnetic resonance.
        J Cardiovasc Magn Reson. 2006; 8: 417-426
        • Ramazzotti A.
        • Pepe A.
        • Positano V.
        • Rossi G.
        • De Marchi D.
        • Brizi M.G.
        • et al.
        Multicenter validation of the magnetic resonance T2* technique for segmental and global quantification of myocardial iron.
        J Magn Reson Imaging. 2009; 30: 62-68
        • Positano V.
        • Pepe A.
        • Santarelli M.F.
        • Scattini B.
        • De Marchi D.
        • Ramazzotti A.
        • et al.
        Standardized T2* map of normal human heart in vivo to correct T2* segmental artefacts.
        NMR Biomed. 2007; 20: 578-590
        • Kawel N.
        • Nacif M.
        • Arai A.E.
        • Gomes A.S.
        • Hundley W.G.
        • Johnson W.C.
        • et al.
        Trabeculated (noncompacted) and compact myocardium in adults: the multi-ethnic study of atherosclerosis.
        Circ Cardiovasc Imaging. 2012; 5: 357-366
        • Stacey R.B.
        • Andersen M.M.
        • St. Clair M.
        • Hundley W.G.
        • Thohan V.
        Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR.
        J Am Coll Cardiol Img. 2013; 6: 931-940
        • Petersen S.E.
        CMR and LV noncompaction: does it matter how we measure trabeculations?.
        J Am Coll Cardiol Img. 2013; 6: 941-943