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Iolated left ventricular non-compaction
 
Published on 2015-02-14 by Elie Chammas
 
 
History and Findings
 

A 22 y Old Female was admitted for Palpitations and Syncope.

•History of Syncope 5 Years ago.

•No Family History of cardiac diseases

•Normal clinical exam

•Normal ECG

•Normal 24 h cardiac Holter

•Late Potentials: Negative

•A cardiac Echocardiography was done showing the images below. The videos are from another patient with the same diagnosis (contributed y Dr. Walid Gharzuddine).

 

Diagnosis: Non Compacting left Ventricular Hypertrophy (NCLVH)

 

 
 
 
video 1 | video 2
 
 
   
 
 
Discussion
 

 

•First Described by echocardiography in 1984

•It is considered as a Non Classified Cardiomyopathy according to the World Health Organization and is an idiopathic cardiomyopathy that occurs as a result of intrauterine arrest of compaction of the myocardial fibers characterized by prominent myocardial trabeculations and deep inter-trabecular recesses, as well as the thickening of the myocardium into two distinct layers (compacted and not compacted). A complicating issue in the diagnosis of this entity is that trabeculations may be noted in many cases of systolic dysfunction and even in athlete hearts. This makes characterisation of the prognosis and management highly variable and cases must be individualized. The reader is referred to a recent review on the subject (ref. 4) as well as to the criteria below (ref.1, 2, and 3).

 

•The Echo Criteria for the diagnosis of the NCLVH are: Segmental Thickening of the LV, two Layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses.

 

 

1. Criteria proposed by Chin et al: Presence of x / y < 0.5, where:

X = distance from the epicardial surface to the trabecular recess; Y = distance from the epicardial surface to the peak of trabeculations.

These criteria are applied to trabeculations of the left ventricular apex with subxiphoid or apical four-chamber views at the end of the diastole.

2. Criteria proposed by Stollberger et al32

• Presence of more than three trabeculations in the left ventricular wall, apical to the papillary muscles, visible in one image plane.

• Intertrabecular spaces, perfused from the ventricular cavity, viewed by color Doppler imaging.

3. Criteria proposed by Jenni et al30,31

• Absence of coexisting cardiac abnormalities.

• Segmental thickening of myocardial wall of left ventricle with two layers: a thin epicardial layer and a thick endocardial layer with prominent trabeculations and deep recesses. The ratio of non-compacted myocardium to compact myocardium at the end of systole is > 2:1;

• The trabeculae are usually located on the apical/lateral, middle/bottom walls of the left ventricle. Most non- compacted segments are hypokinetic.

• The flow between the intertrabecular recesses can be identified by using the color Doppler method (Figure 1).

 

Natural history and course: Given the variability in diagnostic criteria Non-compaction may be asymptomatic or may carry a high morbidity and mortality from ventricular arrhythmias and sudden death to thromboembolism and heart failure.

 

• Treatment: classical treatments for heart failure including anticoagulation, implantable defibrillator and  heart Transplantation in the advanced stages.

 

References:

1.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;86:666-71

 

2. Stöllberger C, Gerecke B, Finsterer J, et-al. Refinement of echocardiographic criteria for left ventricular noncompaction. Int J Cardiol. 2011.

 

3. Chin TK, Perloff JK, Williams RG, et-al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990;82:507-13.

 

4. Gati S. PhD,  Rajani R. MD, Carr-White G. PHD, Chambers J. PhD . Adult Left Ventricular Noncompaction Reappraisal of Current Diagnostic Imaging Modalities. JACC: CARDIOVASCULAR IMAGING, VOL. 7, NO. 12, 2014:1266–75

 

 
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