Noncompaction cardiomyopathy left ventricular dysfunction
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Pathophysiology |
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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 has been documented on thallium 201 scintigraphy[9], on
Because of the prominent, numerous trabeculae, subendocardial ischemia may result from isometric contraction of the endocardium and myocardium within the deep intertrabecular recesses.
Subendocardial perfusion defects have been described in INVM using cardiac magnetic resonance imaging (MRI).25 Positron emission tomography (PET)26 and scintigraphy with thallium-20123 have demonstrated transmural perfusion defects correlating with areas of noncompacted myocardium in INVM. The presence of ischemic subendocardial lesions discovered during postmortem analysis of individuals with INVM lends support to the theory that coronary microcirculatory abnormalities may play a key role in its pathophysiology. Diminished coronary flow reserve has been demonstrated by PET in both noncompacted and compacted segments of myocardium in INVM.27 Epicardial coronary stenosis is unlikely to explain the decreased coronary flow reserve in patients with INVM.1,3,13,26 Impaired microvascular function may account for the contractile dysfunction. Junga et al26 suggested that altered perfusion and coronary flow reserve in INVM may be related to failure of the coronary microcirculation to grow with the increasing ventricular mass, compression of the intramural coronary bed by the hypertrophied myocardium, or both processes.
Restriction
A restrictive pattern of filling can be observed on cardiac catheterization[10][11].
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.
- ↑ 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.