Iatrogenic cyanosis and clubbing: 25 years of chronic hypoxia after the repair of an atrial septal defect

A case of sinus venosus atrial septal defect repair of the inferior vena caval type with an unintentional diversion of the inferior vena caval blood to the left atrium is reported. Long-standing, anatomical, right-to-left shunting with cyanosis and hypoxia are associated with systemic and cerebrovascular complications. Cardiac risks depend on the presence or absence of pulmonary hypertension, the associated hematological abnormalities and the degree of anatomical, right-to-left shunting. Cardiac magnetic resonance imaging clarified the etiology of the unexplained cyanosis and delineated the surgical anatomy.