Long-term problems after cavopulmonary anastomosis: diagnosis and management

After nearly three decades, the "Fontan-like" surgical approach for tricuspid atresia and other forms of functional univentricular heart disease have substantially improved life expectancy and functional status of these patients. However, over the years, complications such as persisting or reoccurring cyanosis, dysrhythmia, thromboembolism, protein-losing enteropathy and exercise intolerance have increasingly been reported. The management of these complications are related to their association with residual or recurrent structural lesions, which can be treated by catheter intervention or surgery, and to pathophysiological sequelae, directly associated to the specific characteristics of "Fontan-like" circulation. The recent modification might reduce this type of complication; however, hard data on this are not available yet.