Ebstein Anomaly in a Neonate with Clinically Significant, Reversible, Idiopathic, Left Ventricular Systolic Dysfunction

Ebstein anomaly is a form of congenital heart disease primarily affecting the tricuspid valve (TV) and right ventricle (RV) in 1 in 20,000 live births. The TV malformation and the clinical presentation are both variable. LV systolic dysfunction is uncommon with Ebstein anomaly. In a review of 106 consecutive patients with Ebstein anomaly at Mayo Clinic between July 1, 2001 and February 28, 2003, the authors reported prevalence of 7% LV systolic dysfunction, 18% LV noncompaction, and 36% LV diastolic dysfunction. However, on a 2017 PubMed review of literature searching for “Ebstein anomaly and Left ventricular systolic dysfunction” we found only one reported case in an adult with documented clinical impact. Here, we report a newborn baby girl with mild Ebstein anomaly who presented with severe respiratory failure and left ventricular systolic dysfunction. In our patient, the LV systolic dysfunction was reversible and of unknown etiology. Significant improvement in her respiratory and cardiac function were attributed to early correct diagnosis and aggressive supportive therapy, which hopefully can also be applied to similar neonates to reduce the mortality and morbidity of Ebstein anomaly.