NONCOMPACTION CARDIOMYOPATHY (NCCMP) is a rare congenital cardiomyopathy characterized
by multiple prominent trabeculations and recesses in the endo/myocardium of a noncompacted
left ventricle. The clinical presentation can be very variable, ranging from an asymptomatic
patient to one with symptoms of extreme congestive heart failure, arrhythmias, and/or
systemic thromboembolic events. Although the pathogenesis of NCCMP remains the subject
of debate, the most likely cause is thought to be arrest during embryologic development
of the endocardium and myocardium.
1
NCCMP has recently been the subject of numerous reports and reviews in the cardiology
literature
2
,
3
,
4
,
5
,
6
but to date has received little attention from anesthesiologists. The authors report
a case of NCCMP in a patient who presented with pre-eclampsia, review the echocardiographic
findings, and discuss the implications for anesthesia and perioperative management.Key words
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Cardiothoracic and Vascular AnesthesiaAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Isolated noncompaction of the myocardium in adults.Mayo Clin Proc. 1997; 72: 26-31
- Isolated noncompaction of left ventricular myocardium: A study of eight cases.Circulation. 1990; 82: 507-513
- Noncompaction of the ventricular myocardium.J Am Soc Echocardiogr. 1999; 12: 859-863
- Isolated noncompaction of the ventricular myocardium: Clinical and molecular aspects of a rare cardiomyopathy.Lab Invest. 2002; 82: 117-122
- Noncompaction of the ventricular myocardium.Circulation. 2004; 109: 2965-2971
- Noncompaction of the left ventricle: A new cardiomyopathy is presented to the clinician.Sao Paulo Med J. 2006; 124: 31-35
- Frequency and location of prominent left ventricular trabeculations at autopsy in 474 normal human hearts: Implications for evaluation of mural thrombi by two-dimensional echocardiography.J Am Coll Cardiol. 1987; 9: 323-326
- Contemporary definitions and classification of the cardiomyopathies: An American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention.Circulation. 2006; 113: 1807-1816
- Genetic analysis in patients with left ventricular noncompaction and evidence for genetic heterogeneity.Mol Genet Metab. 2006; 88: 71-77
- Novel gene locus for autosomal dominant left ventricular noncompaction maps to chromosome 11p15.Circulation. 2004; 109: 2720-2723
- Clinical features of isolated ventricular noncompaction in adults: Long-term clinical course, echocardiographic properties, and predictors of left ventricular failure.J Card Fail. 2006; 12: 726-733
- Noncompaction of the left ventricle: Primary cardiomyopathy with an elusive genetic etiology.Curr Opin Pediatr. 2007; 19: 619-627
- Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: A step towards classification as a distinct cardiomyopathy.Heart. 2001; 86: 666-671
- 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
- Prevalence and characteristics of left ventricular noncompaction in a community hospital cohort of patients with systolic dysfunction.Echocardiography. 2008; 25: 8-12
Article info
Publication history
Published online: September 01, 2008
Identification
Copyright
© 2009 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
