Ebstein’s anomaly (EA) is a rare cyanotic congenital heart disease, affecting 1/200,000 live births and accounts for <1% of congenital heart diseases.1 First described by Wilhelm Ebstein in 18662, the anomaly consists of downward displacement of the septal and posterior leaflet of tricuspid valve resulting in atrialization of the right ventricle and tricuspid regurgitation. The resulting functional impairment of right ventricle retard the forward flow of blood through right side of the heart, thereby causing a hypoplastic Right ventricle(RV), or RV outflow tract obstruction. This creates an accessory conduction pathways resulting in tachyarrythmias, pulmonary hypertension, intracardiac shunting.3 Acute decompensated heart failure and sudden collapse are the most common cause of death.4
There is a wide spectrum of severity of disease with patients ranging from those who are asymptomatic, surgically treated or diagnosed incidentally later in adulthood. Although Ebstein’s anomaly is not generally associated with other congenital defects, patients may occasionally require surgery for other comorbid conditions or for emergency surgery as happened in our patient.
We describe the management of a patient who was a known case of Ebstein’s anomaly (repaired at 2009) posted for emergency laparotomy.
Ebstein’s anomaly; Anesthesia; Congenital heart disease; laparotomy.