Atrial septal defect coexistent with Sjögren's syndrome

Pulmonary hypertension is frequently associated with atrial septal defect and various connective tissue disorders. This case describes a 74-year-old woman who presented with symptoms of heart failure and concomitant involvement of salivary glands and keratoconjunctivitis. An echocardiogram demonstrated ostium secundum atrial septal defect with left to right shunt and severe pulmonary hypertension. Laboratory investigations confirmed the diagnosis of Sjögren's syndrome (SS) with positive anti-nuclear factor and centromere SS-A/Ro pattern. Anti-Ro (SS-A) was found positive. Atrial septal defect was closed through transcatheter route with significant improvement in clinical outcome. This case report suggests a possible association of atrial septal defect with primary Sjögren's syndrome in an adult patient.