Noncompaction cardiomyopathy echocardiography: Difference between revisions
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==Overview== | ==Overview== | ||
On echocardiography there is ventricular hypertrophy with deep recesses which tend to be located apically <ref>Weiford BC, Subbarao VD, Mulhern KM, Noncompaction of the ventricular myocardium. Circulation 109 (24): 2965-71 2004</ref>. Unless there is an awareness of the syndrome, the diagnosis is often missed in up to 89% of children <ref>Ichida F, Hanamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999; 34: 233–240.</ref>. Likewise, the time from onset of symptoms to the diagnosis was more than 3 years in adult patients<ref>.Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997; 72: 26–31.</ref>. | |||
==Conditions that Noncompaction Cardiomyopathy should be Distinguished from on Echocardiography== | |||
*Normal varaint with < 3 prominent trabeculations | |||
*Left ventricular thrombus | |||
*An apical variant of [[hypertrophic obstructive cardiomyopathy]] | |||
*[[Endocardial fibroelastosis]] | |||
*[[Dilated cardiomyopathy]] | |||
*[[Arrhythmogenic right ventricular dysplasia]] | |||
*Metastases to the heart | |||
==Transthoracic Echocardiographic Findings<ref>Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. 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.</ref>== | |||
*There are deep trabeculations in the ventricular wall | |||
*There is systolic dysfunction with an average LVEF of 33% | |||
*It is notable that there is hypokinesis in both the noncompacted as well as the compacted segments. It has been speculated that this is due to the microcirculatory defect observed in these patients. | |||
*There was diastolic dysfunction in all 17 patients in one series, with 36% of these patients having a restrictive filling pattern. | |||
*The left ventricular wall is thick with a two layered appearance | |||
*The epicardial layer is compacted and thin and the endocardial layer is non-compacted and thick | |||
*The ratio of the non-compacted endocardial layer to the epicardial layer is > 2 in adults and >1.4 in children | |||
*41% of patients will have involvement of the RV apex | |||
==Role of Transesophageal and Contrast Echocardiography in Improving Sensitivity and Specificity of the Diagnosis of Noncompaction Cardiomyopathy== | |||
Transesophageal echocardiography <ref> | |||
Maltagliati A, Pepi M. Isolated noncompaction of the myocardium: multiplane transesophageal echocardiography diagnosis in an adult. J Am Soc Echocardiogr. 2000; 13: 1047–1049. | |||
</ref> and contrast echocardiography <ref> | |||
Koo BK, Choi D, Ha J, et al. Isolated noncompaction of the ventricular myocardium: contrast echocardiographic findings and review of the literature. Echocardiography. 2002; 19: 153–156. | |||
</ref>have been advocated as methods for improving the sensitivity and specificity of detecting noncompaction cardiomyopathy. | |||
==References== | ==References== | ||
Latest revision as of 07:01, 9 December 2019
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Noncompaction Cardiomyopathy Microchapters |
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Pathophysiology |
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Differentiating Noncompaction Cardiomyopathy from other Diseases |
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Diagnosis |
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Treatment |
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Noncompaction cardiomyopathy echocardiography On the Web |
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Risk calculators and risk factors for Noncompaction cardiomyopathy echocardiography |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
On echocardiography there is ventricular hypertrophy with deep recesses which tend to be located apically [1]. Unless there is an awareness of the syndrome, the diagnosis is often missed in up to 89% of children [2]. Likewise, the time from onset of symptoms to the diagnosis was more than 3 years in adult patients[3].
Conditions that Noncompaction Cardiomyopathy should be Distinguished from on Echocardiography
- Normal varaint with < 3 prominent trabeculations
- Left ventricular thrombus
- An apical variant of hypertrophic obstructive cardiomyopathy
- Endocardial fibroelastosis
- Dilated cardiomyopathy
- Arrhythmogenic right ventricular dysplasia
- Metastases to the heart
Transthoracic Echocardiographic Findings[4]
- There are deep trabeculations in the ventricular wall
- There is systolic dysfunction with an average LVEF of 33%
- It is notable that there is hypokinesis in both the noncompacted as well as the compacted segments. It has been speculated that this is due to the microcirculatory defect observed in these patients.
- There was diastolic dysfunction in all 17 patients in one series, with 36% of these patients having a restrictive filling pattern.
- The left ventricular wall is thick with a two layered appearance
- The epicardial layer is compacted and thin and the endocardial layer is non-compacted and thick
- The ratio of the non-compacted endocardial layer to the epicardial layer is > 2 in adults and >1.4 in children
- 41% of patients will have involvement of the RV apex
Role of Transesophageal and Contrast Echocardiography in Improving Sensitivity and Specificity of the Diagnosis of Noncompaction Cardiomyopathy
Transesophageal echocardiography [5] and contrast echocardiography [6]have been advocated as methods for improving the sensitivity and specificity of detecting noncompaction cardiomyopathy.
References
- ↑ Weiford BC, Subbarao VD, Mulhern KM, Noncompaction of the ventricular myocardium. Circulation 109 (24): 2965-71 2004
- ↑ Ichida F, Hanamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999; 34: 233–240.
- ↑ .Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997; 72: 26–31.
- ↑ Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. 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.
- ↑ Maltagliati A, Pepi M. Isolated noncompaction of the myocardium: multiplane transesophageal echocardiography diagnosis in an adult. J Am Soc Echocardiogr. 2000; 13: 1047–1049.
- ↑ Koo BK, Choi D, Ha J, et al. Isolated noncompaction of the ventricular myocardium: contrast echocardiographic findings and review of the literature. Echocardiography. 2002; 19: 153–156.