The first reported case of non-compacted cardiomyopathy in a preterm infant with Ebstein's anomaly.

Non-compaction of the ventricular myocardium is a congenital dysfunction of ventricular morphogenesis caused by the arrest of normal embryogenesis of the ventricles. It is considered as a rare disease and can involve both ventricles but commonly the left ventricle. It is classified into isolated non-compaction of the ventricles and non-compaction associated with other congenital heart defects. Its association with Ebstein's anomaly is very rare. We are reporting a rare case of severe heart failure and complex cardiac anomaly including biventricular non-compaction cardiomyopathy, Ebstein's anomalies and large patent ductus arteriosus with severe persistent pulmonary hypertension in a 31-weeks-old preterm infant. The infant recovered smoothly and continued to be asymptomatic and off medications throughout his 2 years follow-up period. Up to our knowledge, this is the youngest and first patient yet reported to have this disease at this age with good recovery.

Clinical and echocardiographic features of Ebstein's malformation in Sudanese patients.

UNLABELLED: Ebstein's malformation is a rare congenital cardiac malformation, accounting for about 0.5% of all congenital cardiac lesions. We report our experience with the anomaly as encountered at the Sudan Heart Centre from July 2004 to April 2005. Diagnosis was based on the echocardiographic demonstration of displacement of the septal leaflet of the tricuspid valve towards the ventricular apex by greater than 8 millimetres per metre squared. RESULTS: In a period of 10 months, we identified 12 patients with the malformation, this number constituting 2% of all patients seen with congenital cardiac disease. The age ranged from 2 weeks to 35 years. Of the patients, half were asymptomatic, while the other half presented with congestive cardiac failure, 4 of these having cyanosis in addition to heart failure. Associated diseases included chronic renal failure, impaired hearing, stunted growth, and developmental delay. Electrocardiographic abnormalities included peaked and tall P waves, seen in four-fifths, an Rsr pattern, first degree atrioventricular block, atrial fibrillation, and Wolf-Parkinson-White syndrome, including the Mahaim pattern of pre-excitation. Associated abnormalities diagnosed echocardiographically included atrial septal defects, prolapse of the leaflets of the mitral valve, left ventricular dysfunction, atrial septal aneurysm, pulmonary valvar stenosis, and pericardial effusion. CONCLUSIONS: Ebstein's malformation was seen four times more frequently at the Sudan Heart Centre when compared to the average frequency reported in the Western literature. Many of the associated diseases encountered in Sudan had not previously been reported.