INTRODUCTION

BIVENTRICULAR NON-COMPACTION ASSOCIATED WITH ATRIAL AND VENTRICULAR SEPTAL DEFECTS

A . ZAROUI, A. FEKI, F. EL AYECH, N. LARBI, MS. MOURALI, R. MECHMECHEService des Explorations Fonctionnelles et de Réanimation Cardiologiques. Hôpital La Rabta. Tunis

CASE REPORT

Seuil

CONCLUSION

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)

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 NVM might be a result of abnormalities in the cardiac conduction system. 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).

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.

[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

Figure 1: Apical view showing the prominent trabeculationsand deep intertrabecular recesses by transthoracic two-

dimensional echocardiography

Figure 2: Ventricular non-compaction on the magnetic resonance imaging

Figure 3: Percutaneous atrial septal defect closure

The patient had a percutaneous atrial septal defect closure.

Magnetic resonance imaging was performed and confirmed the same findings. (Figure 2 )

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