The American Heart Hospital Journal


color Doppler evidence of deep intertrabecular recesses.4 authors use only the first three criteria,5


Some although others have


As seen with our patient, standard 2D echocardiography may be non-diagnostic if the intertrabecular recesses cannot be demonstrated by color Doppler, thus there remains the possibility of apical hypertrophy instead of IVNC. As seen in Figure 3, the use of an echocardiographic contrast agent may allow visualization of the recesses, clarifying the diagnosis, or may demonstrate that color Doppler signals thought to be trabeculations are coronary flow rather than intertrabecular recesses.7


demonstrated a high sensitivity and specificity for IVNC compared with other cardiomyopathies if all four criteria are used.6


Cardiac magnetic resonance (CMR) imaging can be used for diagnosis; however, the criteria are somewhat different. Rather than the usual nine-segment model of the left ventricle used in echocardiography, the CMR diagnosis is based on a 17-segment model and suggests a ratio of non-compacted to compacted myocardium of 2.3 in diastole is the most accurate criterion for making the diagnosis of IVNC.8


There are also series describing diagnosis made on the basis of ventriculography.


Genetic mutations are associated with non-compaction; however, there is not a single gene mutation yet identified that accounts for the majority of disease. Additionally, while


1. Engberding R, Bender F, Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids, Am J Cardiol, 1984;53(11):1733–4.


2. Nugent AW, Daubeney PE, Chondros P, et al., The epidemiology of childhood cardiomyopathy in Australia, N Engl J Med, 2003;348(17): 1639–46.


3. Oechslin EN, Attenhofer Jost CH, 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(2): 493–500.


4. Jenni R, Oechslin EN, van der Loo B, Isolated ventricular non- compaction of the myocardium in adults, Heart, 2007;93(1):11–5.


5. Lofiego C, Biagini E, Pasquale F, et al., Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction, Heart, 2007;93(1):65–71.


Echocardiography Clinic


by definition there are not other cardiac disorders in patients with IVNC, there are some data to suggest there may be genetic commonality between IVNC and hypertrophic cardiomyopathy (HCM).9


There is also increasing recognition


of a non-compaction phenotype in other disorders, including HCM and congenital heart disease (CHD).


The long-term outcomes are generally poor in symptomatic patients. One series described thromboembolic events in 24% of patients and ventricular tachycardia in over 40% when followed for just over three years.3


Adult patients are


treated with usual heart failure therapies, but consideration for anticoagulation should be a standard part of the treatment algorithm. There are not sufficient data to suggest using different criteria for ICD implantation than suggested by current guidelines. While symptomatic patients have significant complications, the prognosis appears better for those patients with evidence of non-compaction but normal left ventricular systolic function.


Our understanding of ventricular non-compaction, the underlying embryologic and genetic abnormalities, the clinical consequences and long-term outcomes and refined diagnostic criteria will continue to improve. Through additional research, we will be better able to distinguish pathology from normal variants, and will be able to refine treatments for those patients with IVNC. n


6. Frischknecht BS, Attenhofer Jost CH, Oechslin EN, et al., Validation of noncompaction criteria in dilated cardiomyopathy, and valvular and hypertensive heart disease, J Am Soc Echocardiogr, 2005;18(8): 865–72.


7. Prada O, Bouzas-Mosquera A, Alvarez-Garcia N, Apical hypertrophic cardiomyopathy or left ventricular non-compaction? Arch Cardiovasc Dis, 2010;103(4):273–4.


8. Petersen SE, Selvanayagam JB, Wiesmann F, et al., Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging, J Am Coll Cardiol, 2005;46(1):101–5.


9. Hoedemaekers YM, Caliskan K, Majoor-Krakauer D, et al., Cardiac beta-myosin heavy chain defects in two families with non-compaction cardiomyopathy: linking non-compaction to hypertrophic, restrictive, and dilated cardiomyopathies, Eur Heart J, 2007;28(22): 2732–7.


Winter 2010


Left Ventricular Non-compaction—When Are Trabeculations Excessive?


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