Noncompaction cardiomyopathy left ventricular dysfunction
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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
Restriction
A restrictive pattern of filling can be observed on cardiac catheterization[9][10].
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.
- ↑ Hook S, Ratliff NB, Rosenkranz E, et al. Isolated noncompaction of the ventricular myocardium. Pediatr Cardiol. 1996; 17: 43–45.