Functionally univentricular heart with systemic venous anomalies: surgical palliation and pulmonary arterial reconstruction with a roll of left atrial appendage.

In this report, we describe a 3-year-old patient with a functionally univentricular heart (UVH), who had a combination of double outlet right ventricle (DORV) along with an unrouteable interventricular communication (VSD), severe infundibular and pulmonary valvar stenosis, and severe left pulmonary artery (LPA) ostial stenosis. This patient also had an interrupted inferior caval vein (IVC) with bilateral superior caval veins (SVC). We were able to undertake a successful Kawashima procedure with interruption of the antegrade pulmonary blood flow, reconstructing the LPA using a pedicled roll of the left atrial appendage (LAA).