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BIVENTRICULAR NON-COMPACTION ASSOCIATED WITH
ATRIAL AND VENTRICULAR SEPTAL DEFECTS
A . ZAROUI, A. FEKI, F. EL AYECH, N. LARBI, MS. MOURALI, R. MECHMECHE
Service des Explorations Fonctionnelles et de Réanimation Cardiologiques. Hôpital La Rabta. Tunis
INTRODUCTION
Noncompaction of the ventricular myocardium (NVM) is a disease of
endomyocardial development characterized by numerous, excessively prominent
ventricular trabeculations and deep recesses, which create characteristic
appearance of disease.
NVM can be isolated or associated with congenital. Assocition with both atrial
and ventricular septal defect is rare.
CASE REPORT
Previously healthy 30-year-old man referred to our department for palpitations
and a heart murmur. The general physical examination was unremarkable. The
auscultation revealed a parasternal holosystolic murmur.
ECG: sinus rhythm and right bundle branch block.
TTE : prominent trabeculations of the apex of both left and right ventricles
compatible with biventricular noncompaction associated with a restrictive
ventricular septal defect and an atrial septum defect ostium secundum. ( Figure 1)
Figure 1: Apical view showing the prominent trabeculations
and deep intertrabecular recesses by transthoracic twodimensional echocardiography
Magnetic resonance imaging was performed and confirmed the same findings.
(Figure 2 )
Figure 3: Percutaneous atrial septal defect closure
The patient had a percutaneous atrial septal defect closure.
DISCUSSION
Myocardial noncompaction is a rare congenital cardiomyopathy secondary to
interruption of the normal myocardial morphogenesis. It occurs more
frequently in males and its prevalence changes from 0.06 to 0.24% and nearly
half of them are familial.
Myocardial noncompaction may result from any interruption during
myocardial morphogenesis. (1) In some familial cases was described the
mechanism considered responsible: a mutation in the G4.5 gene of the Xq28
chromosome region.
There are controversies regarding the right ventricle noncompaction due to the
normally trabeculated shape of its walls.
The three major clinical manifestations of this disease are as follows: heart
failure, ventricular arrhythmias and thromboembolism. Arrhythmogenesis in
Seuilbe a result of abnormalities in the cardiac conduction system.
NVM might
This assumption is substantiated by the finding of Purkinje's fibers as
components of false tendons, which are found frequently in NVM (2). In
addition, it is well known that ventricular conduction abnormalities may occur
later in the course of the NVM due to the development of more severe
endocardial fibroelastosis and sympathetic nerve dysfunction in areas of NMV
The cardiac insufficiency is both systolic and diastolic and in the greatest
number of cases is severe. Especially in the cases of NMV associated with
other cardiac diseases the noncompaction is frequently misdiagnosed..
The quantitative evaluation for the diagnosis of NVM could be done by
determining the ratio of maximal thickness of the noncompacted to compacted
layers (measured at end systole in a parasternal short axis view), with a ratio >
2 diagnostic of NVM. (3)
Both in INMV and NMV the prognosis is bad, and the mortality is high. Until
now, the treatment is the classical one for heart failure, and if the patients are
diagnosed in the early phases we can consider heart transplant (until now there
are few cases described in the literature).
CONCLUSION
Biventricular non compaction is a rare disease, often late diagnosed. Its
prompt recognition is extremely important because of its high mortality and
morbidity due to progressive heart failure, thromboembolism and malignant
arrhythmias
REFERENCES
Figure 2: Ventricular non-compaction on the magnetic
resonance imaging
[1] Muzzarelli S, Left ventricular noncompaction: is it really isolated. Eur J Echocardiogr 2008 ;9 :321–2
[2] Richardson P. Report of the 1995WHO. Circulation 1996;93 : 841–2
[3] Jenni R. Echocardiographic and pathoanatomical. Heart 2001 ; 86 : 666–71