Ebstein anomaly

Abstract

Clinical, electrocardiographic, roentgenologic, hemodynamic and cardioangiographic data in 29 cases of Ebstein anomaly were analysed. This unusual congenital anomaly was characterized clinically by peripheral cyanosis with only a mild degree of oxygen unsaturation in arterial blood, marked enlargement of heart, grade II-IV/VI systolic murmur and low pitched diastolic murmur over the apical region, and a decreased and splitting second heart sound. 80% of them had typical RBBB and tall P waves implying right atrial enlargement in ECGs. Right heart catheterization was performed in 18 cases. The findings are considered to be helpful in diagnosis. The functional classification correlated well with the hemodynamic findings. Patients with this anomaly have a poor prognosis. Most of them died of congestive heart failure or as postoperative deaths.