Case Report

New Onset Heart Failure in a 29-year-old: A Case Report of Isolated Left Ventricular Noncompaction

Authors: Ervin H. Fox, MD, MPH, Michael L. Wood, MD, John Trotter, MD, Charles Moore, MD

Abstract

A previously healthy 29-year-old patient presented with new onset congestive heart failure. Based on findings on transthoracic echocardiogram (TTE) and cardiac magnetic resonance imaging (MRI) at an outside center, the patient was diagnosed as having a dilated cardiomyopathy with structural abnormalities in the ventricular septum and left ventricular (LV) apex suspicious for myocardial tumor. After referral to our center for further management, repeat TTE revealed findings characteristic of left ventricular noncompaction (LVNC) with severely depressed overall LV systolic function. Review of the outside cardiac MRI supported the diagnosis of LVNC. Final management consisted of traditional medical therapy for congestive heart failure, an implantable cardiac defibrillator (ICD), warfarin anticoagulation for the prevention of thromboembolism and referral for cardiac transplant.


Key Points


* Isolated left ventricular noncompaction (LVNC) is a rare congenital cardiomyopathy that appears to result from the intrauterine arrest of endomyocardial morphogenesis that occurs in week 5 to 8 of fetal life.


* Its common presentation involves heart failure, ventricular arrhythmias and thromboembolic events.


* Familial occurrence is common in the reported cases of LVNC, reaching up to 50% of cases in one study.


* Echocardiographic features include the presence of a thin (compacted) epicardium, the presence of a thick, spongy endocardial (noncompacted) surface with extensive trabeculation and evidence of communication between the left ventricular cavity and the deep intertrabecular recesses on low scale color flow Doppler.


* There is no specific treatment for LVNC and therapeutic measures are directed at dealing with the patient's symptoms (heart failure, cardiac arrhythmias, thromboembolic events) and consideration for defibrillator placement and/or cardiac transplantation.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Varnava AM. Isolated non-compaction: a distinct cardiomyopathy? Heart 2001;86:599–600.
 
2. Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation 1996;93:841–842.
 
3. Tamborini G, Pepi M, Celeste F, et al. Incidence and characteristics of left ventricular false tendons and trabeculation in the normal and pathologic heart by second harmonic echocardiography. J Am Soc Echocardiogr 2004;17:367–374.
 
4. Rigopoulos A, Rizos IK, Aggeli C, et al. Isolated left ventricular noncompaction: an unclassified cardiomyopathy with severe prognosis in adults. Cardiology 2002;98:25–32.
 
5. Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy.Heart 2001;86:666–671.
 
6. Ozkutlu S, Ayabakan C, Celiker A. Noncompaction of ventricular myocardium: a study of twelve patients. J Am Soc Echocardiogr 2002;15:1523–1528.
 
7. Pagnatelli RH, McMahon CJ, Dreyer WJ, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 2003;108:2672–2678.
 
8. Kurosaki K, Ikeda U, Hojo Y, et al. Familial isolated noncompaction of the left ventricular myocardium. Cardiology 1999;91:69–72.
 
9. Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular noncompaction of the left ventricular myocardium: a study of eight cases. Circulation 1990;82:507–513.
 
10. Oechlin 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 Cardiol2000;36:493–500.
 
11. Bleyl SB, Mumford BR, Brown-Harrison MC, et al. Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals. Am J Genet1997;72:257–265.
 
12. Wood MJ, Picard MH. Utility of echocardiography in the evaluation of individuals with cardiomyopathy. Heart 2004;90:707–712.
 
13. Ichida F, Hammamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium. J Am Coll Cardiol 1999;34:233–240.
 
14. Agmon Y, Connolly HM, Olson LJ, et al. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr 1999;12:859–863.
 
15. Ali SKM, Du Plessis J, Godman M. Non-compaction of ventricular myocardium: clinical and echocardiographic features of 8 cases. Heart 2002;88:iv29.
 
16. Ali SK, Omran AS, Najm H, et al. Noncompaction of the ventricular myocardium associated with mitral regurgitation and preserved ventricular systolic function. J Am Soc Echocardiogr 2004;17:87–90.
 
17. Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997;72:26–31.