Off-pump total cavopulmonary connection in a patient with hypoplastic right pulmonary artery and right lung secondary to kyphoscoliosis.

The last-stage palliation of patients with single ventricle physiology hearts is Fontan procedure and its modifications that can be performed with or without cardiopulmonary bypass in the current era. Pulmonary vasculature and status of the lungs are critically important for the success of the operation. In this report, we present a 10-year-old patient with single ventricle heart and severely hypoplastic unilateral pulmonary artery and respective lung due to kyphoscoliosis who underwent extracardiac Fontan procedure without cardiopulmonary bypass, successfully.