An unusual fenestration in single-stage Fontan operation.

Fontan operation and importance of fenestration in the treatment of unusual and complex forms of double outlet right ventricle (DORV) are well established. Nonetheless, rarely, the creation of fenestration becomes challenging in complex morphologies. We present one such child with situs solitus, dextrocardia, DORV, hypoplastic right ventricle, large ventricular septal defect, severe pulmonic stenosis, extremely small right atrium and left juxtaposed atrial appendages, who underwent Fontan operation. We created an unusual fenestration between left pulmonary artery and juxtaposed right atrial appendage on the left side, due to anatomic complexity. Short-term results are encouraging.