Answer for BIR CoW 27 Sep 2020
NON ISCHAMEIC DILATED CARDIOMYOPATHY WITH NONCOMPACTION PHENOTYPE
Findings
Significant dilatation of left ventricle is observed The ratio of non-compacted to compacted myocardium was increased (2.82) in end diastole(Peterson Method). Non compaction is predominantly seen in the lateral wall of mid body and apex. Cine imaging showed hypokinesia of left ventricle. Ejection fraction was reduced (28%) Native T1 mapping revealed significantly increased T1 relaxation values (1450-1490) Post contrast delayed enhancement imaging showed no significant abnormal myocardial hyperenhancement reflecting myocardial fibrosis/ scar Relative focal patchy delayed hyperenhancement noted in the trabeculae of non compacted myocardium. The above findings were consistent with diagnosis of Dilated Cardiomyopathy with hypertrabeculations and Non Compaction Phenotype. The absence of subendocardial /transmural delayed hyperenhancement excluded ischameic etiology while absent midmyocardial hyperenhancement excluded post myocarditic forms . No pertinent history for secondary causes were present
Discussion
Dilated cardiomyopathy is characterized by left ventricular or biventricular dilatation with severely impaired systolic function in the absence of abnormal loading conditions (i.e., hypertension, valve disease, etc.) or ischaemic heart disease. Cardiac MRI reveals the dilation of the left ventricle and significantly reduced ejection fraction. Adult patients with DCM revealed myocardial hypertrabeculations satisfying previously reported criteria for diagnosis of noncompacted cardiomyopathy The presence and amount of Left ventricular noncompaction (LVNC) myocardium measured by CMR did not correlate with disease severity and did not predict subsequent major adverse cardiac events (MACE) Mid or subepicardial striae of LGE represent the typical feature of postmyocarditic forms of disease in which ventricular dilatation either may occur acutely after the inflammation as the consequence of the direct damage of the cardiomyocytes by the etiologic agent causing extensive myocardial injury. Depiction of active myocardial inflammation in DCM is important as these patients may benefit and favourably respond to immunomodulatory therapy. Transmural or subendocardial LE pattern in DCM strongly suggests the presence of a previous myocardial infarction even in the absence of remarkable coronary angiographic abnormalities . Myocardial T1 mapping is a noninvasive modality to assess diffuse myocardial fibrosis and Native myocardial T1 values can be used to discriminate normal and diffusely diseased myocardium. Left ventricular non compaction (LVNC) is a cardiomyopathy characterized by prominent left ventricular trabeculae, deep intertrabecular recesses, and thin compacted layer. Left ventricular noncompaction (LVNC) is a relatively new entity. It is characterized by trabeculated myocardium with adjacent deep intertrabecular recesses communicating with the LV cavity In LVNC they are generally located at apex or in midventricular segments In LVNC, areas of trabecular and subendocardial delayed contrast enhancement can be appreciated and are related to the presence of subendocardial and trabecular fibrosis and fibroelastosis. Peterson criteria for left ventricular Non Compaction: Visual appearance of two distinct myocardial layers—a compacted epicardial layer and a noncompacted endocardial layer Presence of marked trabeculations and deep intertrabecular recesses within the noncompacted layer Noncompacted-to-compacted myocardial ratio greater than 2.3 as measured in end-diastole. The prognosis of Non Ischaemic cardiomyopathy depends on presence and amount of myoardial delayed hyperenhancement . However studies have shown that prognosis is not influenced by myocardial hypertrabeculations .
REFERENCES
1JACC Cardiovasc Imaging. 2015 Aug;8(8):934-46. doi: Prognostic Impact of Hypertrabeculation and Noncompaction Phenotype in Dilated Cardiomyopathy: A CMR Study.Amzulescu MS1, Rousseau MF1, Ahn SA1, Boileau L1, de Meester de Ravenstein C1, Vancraeynest D1, Pasquet A1, Vanoverschelde JL1, Pouleur AC1, Gerber BL
2.Role of Cardiac Magnetic Resonance in the Evaluation of Dilated Cardiomyopathy: Diagnostic Contribution and Prognostic Significance Marco Francone ISRN Radiology http://dx.doi.org/10.1155/2014/365404
3.MRI of Cardiomyopathy : Elena Belloni Francesco De Cobelli Antonio Esposito Renata Mellone Gianluca Perseghin Tamara Canu Alessandro Del Maschio DOI:10.2214/AJR.07.3997
4.Cardiac MR Imaging of Nonischemic Cardiomyopathies: Imaging Protocols and Spectra of Appearances David H. O’Donnell, Suhny Abbara, Vithaya Chaithiraphan, Kibar Yared, Ronan P. Killeen, Ramon Martos, David Keane, Ricardo C. Cury, Jonathan D. Dodd https://doi.org/10.1148/radiol.11100284
Findings
Significant dilatation of left ventricle is observed The ratio of non-compacted to compacted myocardium was increased (2.82) in end diastole(Peterson Method). Non compaction is predominantly seen in the lateral wall of mid body and apex. Cine imaging showed hypokinesia of left ventricle. Ejection fraction was reduced (28%) Native T1 mapping revealed significantly increased T1 relaxation values (1450-1490) Post contrast delayed enhancement imaging showed no significant abnormal myocardial hyperenhancement reflecting myocardial fibrosis/ scar Relative focal patchy delayed hyperenhancement noted in the trabeculae of non compacted myocardium. The above findings were consistent with diagnosis of Dilated Cardiomyopathy with hypertrabeculations and Non Compaction Phenotype. The absence of subendocardial /transmural delayed hyperenhancement excluded ischameic etiology while absent midmyocardial hyperenhancement excluded post myocarditic forms . No pertinent history for secondary causes were present
Discussion
Dilated cardiomyopathy is characterized by left ventricular or biventricular dilatation with severely impaired systolic function in the absence of abnormal loading conditions (i.e., hypertension, valve disease, etc.) or ischaemic heart disease. Cardiac MRI reveals the dilation of the left ventricle and significantly reduced ejection fraction. Adult patients with DCM revealed myocardial hypertrabeculations satisfying previously reported criteria for diagnosis of noncompacted cardiomyopathy The presence and amount of Left ventricular noncompaction (LVNC) myocardium measured by CMR did not correlate with disease severity and did not predict subsequent major adverse cardiac events (MACE) Mid or subepicardial striae of LGE represent the typical feature of postmyocarditic forms of disease in which ventricular dilatation either may occur acutely after the inflammation as the consequence of the direct damage of the cardiomyocytes by the etiologic agent causing extensive myocardial injury. Depiction of active myocardial inflammation in DCM is important as these patients may benefit and favourably respond to immunomodulatory therapy. Transmural or subendocardial LE pattern in DCM strongly suggests the presence of a previous myocardial infarction even in the absence of remarkable coronary angiographic abnormalities . Myocardial T1 mapping is a noninvasive modality to assess diffuse myocardial fibrosis and Native myocardial T1 values can be used to discriminate normal and diffusely diseased myocardium. Left ventricular non compaction (LVNC) is a cardiomyopathy characterized by prominent left ventricular trabeculae, deep intertrabecular recesses, and thin compacted layer. Left ventricular noncompaction (LVNC) is a relatively new entity. It is characterized by trabeculated myocardium with adjacent deep intertrabecular recesses communicating with the LV cavity In LVNC they are generally located at apex or in midventricular segments In LVNC, areas of trabecular and subendocardial delayed contrast enhancement can be appreciated and are related to the presence of subendocardial and trabecular fibrosis and fibroelastosis. Peterson criteria for left ventricular Non Compaction: Visual appearance of two distinct myocardial layers—a compacted epicardial layer and a noncompacted endocardial layer Presence of marked trabeculations and deep intertrabecular recesses within the noncompacted layer Noncompacted-to-compacted myocardial ratio greater than 2.3 as measured in end-diastole. The prognosis of Non Ischaemic cardiomyopathy depends on presence and amount of myoardial delayed hyperenhancement . However studies have shown that prognosis is not influenced by myocardial hypertrabeculations .
REFERENCES
1JACC Cardiovasc Imaging. 2015 Aug;8(8):934-46. doi: Prognostic Impact of Hypertrabeculation and Noncompaction Phenotype in Dilated Cardiomyopathy: A CMR Study.Amzulescu MS1, Rousseau MF1, Ahn SA1, Boileau L1, de Meester de Ravenstein C1, Vancraeynest D1, Pasquet A1, Vanoverschelde JL1, Pouleur AC1, Gerber BL
2.Role of Cardiac Magnetic Resonance in the Evaluation of Dilated Cardiomyopathy: Diagnostic Contribution and Prognostic Significance Marco Francone ISRN Radiology http://dx.doi.org/10.1155/2014/365404
3.MRI of Cardiomyopathy : Elena Belloni Francesco De Cobelli Antonio Esposito Renata Mellone Gianluca Perseghin Tamara Canu Alessandro Del Maschio DOI:10.2214/AJR.07.3997
4.Cardiac MR Imaging of Nonischemic Cardiomyopathies: Imaging Protocols and Spectra of Appearances David H. O’Donnell, Suhny Abbara, Vithaya Chaithiraphan, Kibar Yared, Ronan P. Killeen, Ramon Martos, David Keane, Ricardo C. Cury, Jonathan D. Dodd https://doi.org/10.1148/radiol.11100284
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!