Noncompaction cardiomyopathy left ventricular dysfunction: Difference between revisions
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==Systolic Dysfunction== | ==Systolic Dysfunction== | ||
Systolic dysfunction in NCC is thought to be due to subendocardial ischemia and microvascular dysfunciton in the absence of epicardial coronary artery disease. Ischemia in the territory of noncompaction has been documented on thallium 201 scintigraphy<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>, on [[positron emission tomography]] ([[PET]]) <ref>Junga G, Kneifel S, Von Smekal A, et al. Myocardial ischemia in children with isolated ventricular non-compaction. Eur Heart J. 1999; 20: 910–916.</ref>, and on MRI imaging <ref>Soler R, Rodriguez E, Monserrat L, et al. MRI of subendocardial perfusion deficits in isolated left ventricular noncompaction. J Comput Assist Tomogr. 2002; 26: 373–375.</ref>. Lesions consistent with subendocardial ischemia have been observed on autopsy. Impaired coronary flow reserve and microcirculatory defects have been observed on a variety of imaging modalities<ref>Jenni R, Wyss CA, Oechslin EN, et al. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol. 2002;39:450–454.</ref>. | Systolic dysfunction in NCC is thought to be due to subendocardial ischemia and microvascular dysfunciton in the absence of epicardial coronary artery disease. Ischemia in the territory of noncompaction has been documented on thallium 201 scintigraphy<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>, on [[positron emission tomography]] ([[PET]]) <ref>Junga G, Kneifel S, Von Smekal A, et al. Myocardial ischemia in children with isolated ventricular non-compaction. Eur Heart J. 1999; 20: 910–916.</ref>, and on MRI imaging <ref>Soler R, Rodriguez E, Monserrat L, et al. MRI of subendocardial perfusion deficits in isolated left ventricular noncompaction. J Comput Assist Tomogr. 2002; 26: 373–375.</ref>. Lesions consistent with subendocardial ischemia have been observed on autopsy. Impaired coronary flow reserve and microcirculatory defects have been observed on a variety of imaging modalities<ref>Jenni R, Wyss CA, Oechslin EN, et al. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol. 2002;39:450–454.</ref>. The basis for the ischemia has been hypothesized to be extrinsic compression of the microvasculature by the hypertrophied muscle and isometric contraction of the myocardium against the trabeculae. | ||
==Restriction== | ==Restriction== | ||
Latest revision as of 19:49, 6 August 2011
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The phenotypic expression of NCC is variable, but two thirds of patients have left ventricular systolic and / or diastolic dysfunction[1][2][3][4][5][6]. Patients may also present with left ventricular restriction. Even if hemodynamic abnormalities were not present at the time of diagnosis, one study from Japan demonstrated that the majority of children developed hemodynamic abnormalities after 17 years of follow-up[7].
Diastolic Dysfunction
Diastolic dysfunction is thought to be due to abnormal relaxation as a result of extensive trabeculation[8].
Systolic Dysfunction
Systolic dysfunction in NCC is thought to be due to subendocardial ischemia and microvascular dysfunciton in the absence of epicardial coronary artery disease. Ischemia in the territory of noncompaction has been documented on thallium 201 scintigraphy[9], on positron emission tomography (PET) [10], and on MRI imaging [11]. Lesions consistent with subendocardial ischemia have been observed on autopsy. Impaired coronary flow reserve and microcirculatory defects have been observed on a variety of imaging modalities[12]. The basis for the ischemia has been hypothesized to be extrinsic compression of the microvasculature by the hypertrophied muscle and isometric contraction of the myocardium against the trabeculae.
Restriction
A restrictive pattern of filling can be observed on cardiac catheterization[13][14].
Mural Thrombus
These patients are at risk of forming mural thrombus in the left ventricle and embolization. As a result, all patients with NCC are treated with prophylactic aspirin.
References
- ↑ 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.
- ↑ Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation. 1990; 82: 507–513.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Agmon Y, Connolly HM, Olson LJ, et al. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr. 1999; 12: 859–863.
- ↑ 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.
- ↑ Junga G, Kneifel S, Von Smekal A, et al. Myocardial ischemia in children with isolated ventricular non-compaction. Eur Heart J. 1999; 20: 910–916.
- ↑ Soler R, Rodriguez E, Monserrat L, et al. MRI of subendocardial perfusion deficits in isolated left ventricular noncompaction. J Comput Assist Tomogr. 2002; 26: 373–375.
- ↑ Jenni R, Wyss CA, Oechslin EN, et al. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol. 2002;39:450–454.
- ↑ 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.
- ↑ Hook S, Ratliff NB, Rosenkranz E, et al. Isolated noncompaction of the ventricular myocardium. Pediatr Cardiol. 1996; 17: 43–45.