© 2015, Wiley Periodicals, Inc. DOI: 10.1111/echo.12862
Echocardiography
LETTER TO THE EDITOR
Unusual Myocardial Late Gadolinium Enhancement in Isolated Noncompaction Cardiomyopathy To the editor, With interest we read the article by Moustafa et al. about a 19-year-old male admitted for heart failure who presented with hypertrabeculation/noncompaction of the left and right ventricles plus late gadolinium enhancement (LGE).1 We have the following comments and concerns. Left ventricular hypertrabeculation (LVHT) in association with LGE is not unusual and has been repeatedly reported.2,3 In a study of 8 LVHT patients, LGE was detected in 5.2 In addition, LGE has been reported in patients with cardiac involvement in a neuromuscular disorder.4 In a study of 12 patients with LVHT, LGE was a discriminator between LVHT and dilated and hypertrophic cardiomyopathy.5 In a study of 47 LVHT patients, LGE was detected in 19 of them.6 In this study, LGE was most frequently located in the ventricular septum.6 The cause of LGE frequently remains elusive but may be due to previous myocardial infarction (subendocardial or transmural LGE),3 dilated cardiomyopathy (increase in LGE from epicardium to endocardium),3 or due to increased wall stress which enhances capillary leakage.3 LGE may also occur as a mid-wall streak, or as a patchy or diffuse abnormality.3 LGE thus not only represents myocardial fibrosis but may be also attributable to other pathomechanisms.3 Interestingly, LGE in LVHT seems to be a phenomenon particularly seen in adult patients.7 LGE in LVHT is a typical phenomenon of the left ventricle and hardly occurs in rightventricular hypertrabeculation.8 In patients with LVHT and LGE, systolic function is usually reduced.2 LVHT plus LGE is associated with a poor outcome.2 LGE in the presented patient was of the subendocardial type and thus attributable to a dilated cardiomyopathy or previous myocardial infarction. Was coronary angiography normal? In addition, it cannot be excluded that LGE in the presented patient represents involvement of the myocardium in a neuromuscular disorder. This is why all LVHT patients with LGE should be referred to the neurologist. As neuromuscular disorders are most frequently associated with LVHT, it is essential to screen not only the index patient but also other family members for the presence or absence of a neuromuscular disorder. As LVHT
is frequently familial, it is also essential that other family members are investigated for LVHT. As LVHT may represent a risk factor for cardiac embolism it would be interesting to know if the patient also had a history of stroke/embolism. As he received oral anticoagulation because of intertrabecular thrombus formation, the authors should mention if the described thrombi resolved upon treatment at follow-up. It would be also interesting to know if LVHT disappeared upon effective heart failure therapy in this patient because disappearance of LVHT has been previously reported. Overall, this interesting case description may profit from a more widespread investigation of the index patient and other family members. Particularly essential is to rule out a neuromuscular disorder and to document if heart failure therapy had a beneficial effect on LVHT extension and if anticoagulation was effective in resolving intertrabecular thrombi. Josef Finsterer, M.D., Ph.D.,* and Sinda Zarrouk-Mahjoub, Ph.D.† *Municipal Hospital Rudolfstiftung, Vienna, Austria †Laboratory of Biochemistry, UR “Human Nutrition and Metabolic Disorders,” Faculty of Medicine, Monastir, Tunisia References 1. Moustafa S, Patton DJ, Al Shanawani M, et al: Unusual myocardial late gadolinium enhancement in isolated noncompaction cardiomyopathy. Echocardiography 2015;32: 400–402. 2. Fazio G, Novo G, Casalicchio C, et al: Left ventricular noncompaction cardiomyopathy in children: Is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction? Int J Cardiol 2009;132:278–280. 3. Alter P, Rupp H: Myocardial fibrosis in left ventricular noncompaction: Is late gadolinium enhancement indeed indicative of fibrosis? Eur J Heart Fail 2011;13:577–578. 4. Giglio V, Puddu PE, Camastra G, et al: Patterns of late gadolinium enhancement in Duchenne muscular dystrophy carriers. J Cardiovasc Magn Reson 2014;16:45. 5. Grothoff M, Pachowsky M, Hoffmann J, 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. 6. Wan J, Zhao S, Cheng H, et al: Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left
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Letter to the Editor
ventricular non-compaction. J Cardiovasc Magn Reson 2013;15:20. 7. Marın Rodrıguez C, Ossaba Velez S, Maroto Alvaro E, et al: Lack of MR late-enhancement in left ventricular non-compaction in infants and young children. Radiologia 2010;52:138–143.
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8. Nucifora G, Aquaro GD, Masci PG, et al: Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. Am J Cardiol 2014;113:142–146.
Echocardiography
LETTER TO THE EDITOR
Unusual Myocardial Late Gadolinium Enhancement in Isolated Noncompaction Cardiomyopathy To the editor, With interest we read the article by Moustafa et al. about a 19-year-old male admitted for heart failure who presented with hypertrabeculation/noncompaction of the left and right ventricles plus late gadolinium enhancement (LGE).1 We have the following comments and concerns. Left ventricular hypertrabeculation (LVHT) in association with LGE is not unusual and has been repeatedly reported.2,3 In a study of 8 LVHT patients, LGE was detected in 5.2 In addition, LGE has been reported in patients with cardiac involvement in a neuromuscular disorder.4 In a study of 12 patients with LVHT, LGE was a discriminator between LVHT and dilated and hypertrophic cardiomyopathy.5 In a study of 47 LVHT patients, LGE was detected in 19 of them.6 In this study, LGE was most frequently located in the ventricular septum.6 The cause of LGE frequently remains elusive but may be due to previous myocardial infarction (subendocardial or transmural LGE),3 dilated cardiomyopathy (increase in LGE from epicardium to endocardium),3 or due to increased wall stress which enhances capillary leakage.3 LGE may also occur as a mid-wall streak, or as a patchy or diffuse abnormality.3 LGE thus not only represents myocardial fibrosis but may be also attributable to other pathomechanisms.3 Interestingly, LGE in LVHT seems to be a phenomenon particularly seen in adult patients.7 LGE in LVHT is a typical phenomenon of the left ventricle and hardly occurs in rightventricular hypertrabeculation.8 In patients with LVHT and LGE, systolic function is usually reduced.2 LVHT plus LGE is associated with a poor outcome.2 LGE in the presented patient was of the subendocardial type and thus attributable to a dilated cardiomyopathy or previous myocardial infarction. Was coronary angiography normal? In addition, it cannot be excluded that LGE in the presented patient represents involvement of the myocardium in a neuromuscular disorder. This is why all LVHT patients with LGE should be referred to the neurologist. As neuromuscular disorders are most frequently associated with LVHT, it is essential to screen not only the index patient but also other family members for the presence or absence of a neuromuscular disorder. As LVHT
is frequently familial, it is also essential that other family members are investigated for LVHT. As LVHT may represent a risk factor for cardiac embolism it would be interesting to know if the patient also had a history of stroke/embolism. As he received oral anticoagulation because of intertrabecular thrombus formation, the authors should mention if the described thrombi resolved upon treatment at follow-up. It would be also interesting to know if LVHT disappeared upon effective heart failure therapy in this patient because disappearance of LVHT has been previously reported. Overall, this interesting case description may profit from a more widespread investigation of the index patient and other family members. Particularly essential is to rule out a neuromuscular disorder and to document if heart failure therapy had a beneficial effect on LVHT extension and if anticoagulation was effective in resolving intertrabecular thrombi. Josef Finsterer, M.D., Ph.D.,* and Sinda Zarrouk-Mahjoub, Ph.D.† *Municipal Hospital Rudolfstiftung, Vienna, Austria †Laboratory of Biochemistry, UR “Human Nutrition and Metabolic Disorders,” Faculty of Medicine, Monastir, Tunisia References 1. Moustafa S, Patton DJ, Al Shanawani M, et al: Unusual myocardial late gadolinium enhancement in isolated noncompaction cardiomyopathy. Echocardiography 2015;32: 400–402. 2. Fazio G, Novo G, Casalicchio C, et al: Left ventricular noncompaction cardiomyopathy in children: Is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction? Int J Cardiol 2009;132:278–280. 3. Alter P, Rupp H: Myocardial fibrosis in left ventricular noncompaction: Is late gadolinium enhancement indeed indicative of fibrosis? Eur J Heart Fail 2011;13:577–578. 4. Giglio V, Puddu PE, Camastra G, et al: Patterns of late gadolinium enhancement in Duchenne muscular dystrophy carriers. J Cardiovasc Magn Reson 2014;16:45. 5. Grothoff M, Pachowsky M, Hoffmann J, 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. 6. Wan J, Zhao S, Cheng H, et al: Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left
723
Letter to the Editor
ventricular non-compaction. J Cardiovasc Magn Reson 2013;15:20. 7. Marın Rodrıguez C, Ossaba Velez S, Maroto Alvaro E, et al: Lack of MR late-enhancement in left ventricular non-compaction in infants and young children. Radiologia 2010;52:138–143.
724
8. Nucifora G, Aquaro GD, Masci PG, et al: Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. Am J Cardiol 2014;113:142–146.
