Left ventricular non-compaction in children
A Case Report
S.Aggoune1; A.Fafa1 ; A.Djermane1 ; L.Atek1 ; A.Naili1 ; L.Kermi1; D.Boukria1 ; H.Maouche1
1: Pediatric department , EPH Belfort
2: Pediatric department ; CHU Bab El Oued
Background and aims
Left ventricular non-compaction (LVNC) is still classiﬁed as an unclassiﬁed cardiomyopathy by the European Society of Cardiology although it is considered as a primary cardiomyopathy by the American Heart Association ; it is morphologically characterised by a two-layered structure of the myocardium, which consists of a thick non-compacted inner layer and a thin compacted outer layer. Some LVNC cases are congenital and the others seem to be acquired.
The aim of this study, is to highlight the diagnostic and therapeutic difficulties of this extremely rare pathology.
Material and methods
2years old girl, was admitted to the emergency department with a history of asthma who had respiratory distress. Physical examination revealed, heart rate was 260 beats per minute tachypnea, respiratory distress, grunting, and difficulty with feeding. venous congestion - Right-sided with hepatomegaly, a low cardiac output : low energy, pallor, cool extremities.
The cardiothoracic index was 71%. The patient’s electrocardiogram demonstrated supra ventricular tachycardia with fine QRS, Dilated cardiomyopathy (FE=41%, FR=19%) was detected
echocardiographic study. Color Doppler examination showed prominent left ventricular spongiform with deep intertrabecular recesses.The patient was hospitalized, and diuretic therapy (furosemide), captopril and digoxine.The persistence of the supra ventricular tachycardia with heart rase at 300 per minute, led us to introduce Amiodarone.
LVNC is a cardiac disease of emerging importance associated with LV dysfunction,arrhythmias, embolic events, and sudden death. Imaging has a key role in its diagnosis. Echocardiography is the first-line diagnostic technique and depicts the main myocardial features of LVNC.