Abstract 2157: Prognosis of Left Ventricular Noncompaction in Paediatric Age: Results of an International Prospective Study Conducted on 130 Children
Methods and Results: In an international multicentre analysis we enrolled a series of 130 children affected by ventricular noncompaction. 12 different centres of paediatric cardiology in 4 continents (Europe, Asia, Africa and North America) have been involved. Enrolled patients were from 0 to 18 years old. The recruitment period lasted 8 months. Each patient was followed in one of the 12 involved centres with at least annual clinical controls. Patients were followed for 60 months. The average age of children at the time of enrolment was 4,77 years. 109 children were younger than 14. 33% of patients had a reduced ejection fraction (EF) at the time of recruiting. During the follow-up 4 children (3%) died for cardiac causes (all of them had a reduced EF at the time of enrolment, < 35%); none died for non-cardiac cause or because of a documented arrhythmic sudden death. No cardiac transplantation was performed. 26 children (20%) complained of episodes of heart failure so a hospitalization was necessary.2 children (1%) (EF 35 and 65% respectively) developed, during the follow-up, complex ventricular arrhythmias; none developed any kind of cardio-embolic episodes. About the cardiac function, the average EF value was 52.4% at recruiting moment and 53.1% (p: 0.78) at the end of the established follow-up. No patient with normal EF showed deterioration in the EF. At the beginning of the follow-up 22 children (17%) have been practicing medical therapy. At the end of the follow-up 42 cases (32%) were treated with medical therapy, chosen and awarded from each single centre according to the guidelines. The ROC analysis showed a moderate predictive power of EF in predicting mortality (0,703), and a poor predictive power of it for arrhythmias (0,230).
Conclusions: By analyzing data we collected, left ventricular noncompac-tion in paediatric age seems to have a good prognosis in 5 years (only 3% of patients with non-compaction died during the follow-up). In our experience the only cause of death was the heart failure