Background. A review of the history and practice of cavopulmonary connections in staging toward Fontan operation and the pathologic experience at Padua University is presented. Methods. Gross and histologic assessment of the heart-lung blocks removed at autopsy in the cases in which the cause of death could be related to a dysfunction of the cavopulmonary anastomosis were performed. Results. The main complications were distortion of the intraatrial tunnel and thrombosis of the pulmonary branches of the cavopulmonary anastomosis. Conclusions. The cavopulmonary anastomosis remains a widespread procedure, both as a preliminary step to a Fontan operation and as an integral part of a Fontan or modified Fontan procedure in all those cardiac malformations characterized by a hypoplastic right or left ventricle in which these ventricles are too small to support the whole circulation.