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The association between interatrial communication and unicuspid mitral valve is extremely unusual. We present a case of 50-year-old woman with ostium secundum atrial septal defect and unicuspid mitral valve with mild mitral regurgitation. The shape, size and borders of the interatrial septal defect and mitral valve were well characterized by 3-D transesophageal echocardiogram.

We present a 23-year-old man with complex CHD, who came to our institution due to moderate exertion dyspnea and fever. He was diagnosed with mitral valve endocarditis and Granulicatella adiacens was isolated. He was treated with antibiotics and discharged as he refused surgery. He came back on August 2019 with progressive increase of abdominal perimeter, lower limbs edema and chest pain. On cardiac examination pericardial rub and III/VI apical holosystolic murmur were found. The transthoracic echocardiogram showed situs solitus, atrioventricular discordance, double-outlet right ventricle with transposition of great arteries, interventricular septal defect, moderate tricuspid regurgitation, vegetations in both leaflets of mitral valve and severe mitral regurgitation, subvalvular pulmonary stenosis and huge pericardial effusion. The patient underwent successful mitral valve replacement and he was discharged in good condition.

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