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NON-COMPACTION OF VENTRICULAR MYOCARDIUM, CLINICAL AND ECHOCARDIOGRAPHIC FEATURES OF 8 CASES

SKM Ali, J duPlessis, MJ GodmanKing Abdulaziz Cardiac Center, Riyadh, Kingdom of Saudi Arabia

Non-compaction of the ventricular myocardium (NVM) is a rare cardiomyopathy characterized by prominent trabeculations and deep intertrabecular recesses. In this study 8 cases of NVM were followed prospectively. The age of presentation ranged from birth to 10 months. Male to female ratio was 1:3. Clinical presentation was variable; one asymptomatic infant (patient 1) was identified incidentally because of associated patent ductus arteriosus. One infant (patient 2) presented with mild congestive heart failure attributed to associated multiple ventricular septal defects and three patients with isolated NVM (patients 3, 4 and 5) presented with heart failure. In a further three patients (patients 6, 7 and 8) NVM was associated with complex cyanotic congenital heart disease. In all patients diagnosis was confirmed echocardiographically by calculating a ratio of non-compacted to compacted layer thickness of 2 or more. In patients 1 and 2 the ventricular function was normal while it was impaired in the others. Short-term outcome reflected the heterogeneous nature of the disease; patient 1 is doing well with a normal ventricular function, patient 2 showed clinical improvement of congestive heart failure. Patient 3 died with severe myocardial dysfunction at the age of 7 days. Patients 4 and 5 are alive with severely impaired function. Patient 6 died with ventricular arrhythmia following a Blalock-Taussing shunt insertion. Patients 7 and 8 are alive with impaired myocardial function.

NVM may not be as rare as is thought. It should be considered in infants presenting with either congenital heart disease or cardiomyopathy in whom the echocardiographic features suggest excessively prominent trabeculations with or without left ventricular dysfunction.

PREDICTION OF SYMPTOM ONSET IN AORTIC STENOSIS: BASELINE AND SERIAL ECHOCARDIOGRAPHY IN 97 PATIENTS

P Das, H Rimington, J ChambersValve Study Group, Guy’s and St Thomas’ Hospitals, London, UK

Background: It remains unclear which echocardiographic variables are …

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