Cor triatriatum

Background

• Congenital Anamoly with heart with three atria
• Either LA or RA can be divided into 2 compartments (cor triatriatum sinistrum vs cor triatriatum dextrum)
• Can be associated with other congenital syndromes including tetralogy of Fallot, double outlet RV, coarctation of aorta, VSD, AV septal defect
• 0.1-0.4% incidence in USA

Clinical Features

• Sinistrum variant most associated with other congenital cardiovascular defects in symptomatic infants
• Can be incidental finding in adults as either isolated finding or in association with PFO, ASD, persistent L superior vena cava

Management

• As associates with major cyanotic or acyanotic congenital heart lesions, mortality can be up to 75% in untreated symptomatic infants