Steli s S Anomaly ; : Presentation and Outcome From Fetus to Adult

Objectives . This study was conducted to investigate the presentation and outcome of patients with Ebstein's anomaly of the tricuspid valve . Background. Ebstein's anomaly may present at any age and has a highly variable clinical course . Previous natural history studies have been based on clinical and angiographic diagnosis and have included mainly older children and adults . Echocardiography, however, has facilitated fetal and neonatal diagnosis so that the natural history needs to be redefined . Metho:lt. We reviewed 220 casesofEbstein's anomaly presenting front fetal to adult life between 1958 and 1991, with I to 34 years of follow-up. Results. The most common presentation in each age group was abnormal routine prenatal scan for fetuses (86%), cyanosis for neonates (74%), heart failure for infants (43%), incidental murmur for children (63%) and arrhythmia for adolescents and adults (42%). Early presentation was frequently associated with other cardiac lesions, usually pulmonary stenosis or atresia. Surgery