Cor triatriatum

Background

• Congenital Anamoly with heart with three atria
• Either LA or RA can be divided into 2 compartments (cor triatriatum sinistrum or 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
• Symptoms depend on size of opening in accessory membrane between the atria
• Symptoms include dyspnea on exertion, orthopnea, easy fatiguability, low exercise tolerance, palpitations
• Increased risk of thrombus formation in LA

Diagnosis

• Murmur
• Elevated JVD
• Hepatomegaly
• Ascites
• Peripheral edema
• Often discovered on cardiac imaging studies (Echo, Right heart cath)

Management

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

References

• MedScape