A INSIDIOUS LINE BETWEEN THALASSEMIA INTERMEDIA AND LEFT VENTRICULAR NON-COMPACTION DISEASE: THE ROLE OF CARDIAC MAGNETIC RESONANCE
(Abstract release date: 05/19/16)
EHA Library. Meloni A. 06/09/16; 135027; PB2127
Dr. Antonella Meloni
Contributions
Contributions
Abstract
Abstract: PB2127
Type: Publication Only
Background
Differentiation of left ventricle non-compaction (LVNC) from hypertrabeculated LV due to a negative heart remodeling in thalassemia intermedia (TI) can depend on the selected cardiac magnetic resonance (CMR) criterion.
Aims
We verified whether the diastolic non-compacted to compacted myocardium (NC/C) ratio criterion could actually discriminate the abnormal trabeculations observed in β-TI from LVNC patients and we compared this diagnostic approach with the CMR criterion suggested by Grotoff M et al. (Eur Rad 2012), which has been reported to be highly sensitive and specific for the LVNC diagnosis.
Methods
CMR images were analyzed in 180 patients with β-TI consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network and 20 patients with proved diagnosis LVNC studied at FTGM MRI Lab in Pisa from 2002 to 2014.The CMR diagnostic criteria applied in β-TI patients were: a modified CMR Petersen’s criterion proposed by Piga et al (Am J Haem 2012) based on a more restrictive ratio of diastolic NC/C >2.5 at a segmental level and the Grothoff's criteria (percentage of trabeculated left ventricular myocardial mass ( LV-MM) ≥25% of global LV mass and a total LV-MMI NC ≥15 g/m2).In the 20 patients with LVNC the final diagnosis was performed based on Grotoff’s criteria and on the clinical/functional criteria for LVNC to further increase the pre-test probability of the disease.
Results
In β-TI patients at least 1 positive NC/C segment was found in 18 patients (10%).Compared with LVNC patients, in 18 β-TI patients the non-compaction areas were less frequent (3.70±2.22 vs 1.62±1.16; P=0.007). The LV-MM NC percentage and LV-MMI NC g/m2 were significantly higher in LVNC than in β-TI patients 27.21±2.45% vs. 10.88±3.96%, P<0.001; 20.35±5.60 % vs.7.30±4.77 %, P<0.001 ).None of the β-TI patients fulfilled the Grothoff's criteria.The table shows the comparison of CMR parameters. LVNC patients had significant lower LV stroke volume index and LV ejection fraction and they had an higher frequency of myocardial fibrosis detected by the LGE technique, although the statistical significance was not reached.
Conclusion
Differentiation of LVNC from hypertrabeculated LV in β-TI patients due to a negative heart remodeling depends on the selected CMR criterion. Based on our data in all β-TI patients with a NC/C ratio >2.5 we suggest to use Grothoff's criteria to improve the specificity of the diagnosis of LVNC.
Session topic: E-poster
Keyword(s): Magnetic resonance imaging, Thalassemia
Type: Publication Only
Background
Differentiation of left ventricle non-compaction (LVNC) from hypertrabeculated LV due to a negative heart remodeling in thalassemia intermedia (TI) can depend on the selected cardiac magnetic resonance (CMR) criterion.
Aims
We verified whether the diastolic non-compacted to compacted myocardium (NC/C) ratio criterion could actually discriminate the abnormal trabeculations observed in β-TI from LVNC patients and we compared this diagnostic approach with the CMR criterion suggested by Grotoff M et al. (Eur Rad 2012), which has been reported to be highly sensitive and specific for the LVNC diagnosis.
Methods
CMR images were analyzed in 180 patients with β-TI consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network and 20 patients with proved diagnosis LVNC studied at FTGM MRI Lab in Pisa from 2002 to 2014.The CMR diagnostic criteria applied in β-TI patients were: a modified CMR Petersen’s criterion proposed by Piga et al (Am J Haem 2012) based on a more restrictive ratio of diastolic NC/C >2.5 at a segmental level and the Grothoff's criteria (percentage of trabeculated left ventricular myocardial mass ( LV-MM) ≥25% of global LV mass and a total LV-MMI NC ≥15 g/m2).In the 20 patients with LVNC the final diagnosis was performed based on Grotoff’s criteria and on the clinical/functional criteria for LVNC to further increase the pre-test probability of the disease.
Results
In β-TI patients at least 1 positive NC/C segment was found in 18 patients (10%).Compared with LVNC patients, in 18 β-TI patients the non-compaction areas were less frequent (3.70±2.22 vs 1.62±1.16; P=0.007). The LV-MM NC percentage and LV-MMI NC g/m2 were significantly higher in LVNC than in β-TI patients 27.21±2.45% vs. 10.88±3.96%, P<0.001; 20.35±5.60 % vs.7.30±4.77 %, P<0.001 ).None of the β-TI patients fulfilled the Grothoff's criteria.The table shows the comparison of CMR parameters. LVNC patients had significant lower LV stroke volume index and LV ejection fraction and they had an higher frequency of myocardial fibrosis detected by the LGE technique, although the statistical significance was not reached.
Conclusion
Differentiation of LVNC from hypertrabeculated LV in β-TI patients due to a negative heart remodeling depends on the selected CMR criterion. Based on our data in all β-TI patients with a NC/C ratio >2.5 we suggest to use Grothoff's criteria to improve the specificity of the diagnosis of LVNC.
Session topic: E-poster
Keyword(s): Magnetic resonance imaging, Thalassemia
Abstract: PB2127
Type: Publication Only
Background
Differentiation of left ventricle non-compaction (LVNC) from hypertrabeculated LV due to a negative heart remodeling in thalassemia intermedia (TI) can depend on the selected cardiac magnetic resonance (CMR) criterion.
Aims
We verified whether the diastolic non-compacted to compacted myocardium (NC/C) ratio criterion could actually discriminate the abnormal trabeculations observed in β-TI from LVNC patients and we compared this diagnostic approach with the CMR criterion suggested by Grotoff M et al. (Eur Rad 2012), which has been reported to be highly sensitive and specific for the LVNC diagnosis.
Methods
CMR images were analyzed in 180 patients with β-TI consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network and 20 patients with proved diagnosis LVNC studied at FTGM MRI Lab in Pisa from 2002 to 2014.The CMR diagnostic criteria applied in β-TI patients were: a modified CMR Petersen’s criterion proposed by Piga et al (Am J Haem 2012) based on a more restrictive ratio of diastolic NC/C >2.5 at a segmental level and the Grothoff's criteria (percentage of trabeculated left ventricular myocardial mass ( LV-MM) ≥25% of global LV mass and a total LV-MMI NC ≥15 g/m2).In the 20 patients with LVNC the final diagnosis was performed based on Grotoff’s criteria and on the clinical/functional criteria for LVNC to further increase the pre-test probability of the disease.
Results
In β-TI patients at least 1 positive NC/C segment was found in 18 patients (10%).Compared with LVNC patients, in 18 β-TI patients the non-compaction areas were less frequent (3.70±2.22 vs 1.62±1.16; P=0.007). The LV-MM NC percentage and LV-MMI NC g/m2 were significantly higher in LVNC than in β-TI patients 27.21±2.45% vs. 10.88±3.96%, P<0.001; 20.35±5.60 % vs.7.30±4.77 %, P<0.001 ).None of the β-TI patients fulfilled the Grothoff's criteria.The table shows the comparison of CMR parameters. LVNC patients had significant lower LV stroke volume index and LV ejection fraction and they had an higher frequency of myocardial fibrosis detected by the LGE technique, although the statistical significance was not reached.
Conclusion
Differentiation of LVNC from hypertrabeculated LV in β-TI patients due to a negative heart remodeling depends on the selected CMR criterion. Based on our data in all β-TI patients with a NC/C ratio >2.5 we suggest to use Grothoff's criteria to improve the specificity of the diagnosis of LVNC.
Session topic: E-poster
Keyword(s): Magnetic resonance imaging, Thalassemia
Type: Publication Only
Background
Differentiation of left ventricle non-compaction (LVNC) from hypertrabeculated LV due to a negative heart remodeling in thalassemia intermedia (TI) can depend on the selected cardiac magnetic resonance (CMR) criterion.
Aims
We verified whether the diastolic non-compacted to compacted myocardium (NC/C) ratio criterion could actually discriminate the abnormal trabeculations observed in β-TI from LVNC patients and we compared this diagnostic approach with the CMR criterion suggested by Grotoff M et al. (Eur Rad 2012), which has been reported to be highly sensitive and specific for the LVNC diagnosis.
Methods
CMR images were analyzed in 180 patients with β-TI consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network and 20 patients with proved diagnosis LVNC studied at FTGM MRI Lab in Pisa from 2002 to 2014.The CMR diagnostic criteria applied in β-TI patients were: a modified CMR Petersen’s criterion proposed by Piga et al (Am J Haem 2012) based on a more restrictive ratio of diastolic NC/C >2.5 at a segmental level and the Grothoff's criteria (percentage of trabeculated left ventricular myocardial mass ( LV-MM) ≥25% of global LV mass and a total LV-MMI NC ≥15 g/m2).In the 20 patients with LVNC the final diagnosis was performed based on Grotoff’s criteria and on the clinical/functional criteria for LVNC to further increase the pre-test probability of the disease.
Results
In β-TI patients at least 1 positive NC/C segment was found in 18 patients (10%).Compared with LVNC patients, in 18 β-TI patients the non-compaction areas were less frequent (3.70±2.22 vs 1.62±1.16; P=0.007). The LV-MM NC percentage and LV-MMI NC g/m2 were significantly higher in LVNC than in β-TI patients 27.21±2.45% vs. 10.88±3.96%, P<0.001; 20.35±5.60 % vs.7.30±4.77 %, P<0.001 ).None of the β-TI patients fulfilled the Grothoff's criteria.The table shows the comparison of CMR parameters. LVNC patients had significant lower LV stroke volume index and LV ejection fraction and they had an higher frequency of myocardial fibrosis detected by the LGE technique, although the statistical significance was not reached.
Conclusion
Differentiation of LVNC from hypertrabeculated LV in β-TI patients due to a negative heart remodeling depends on the selected CMR criterion. Based on our data in all β-TI patients with a NC/C ratio >2.5 we suggest to use Grothoff's criteria to improve the specificity of the diagnosis of LVNC.
Session topic: E-poster
Keyword(s): Magnetic resonance imaging, Thalassemia
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