Lutembacher's syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement.

Lutembacher's syndrome is a rare clinical combination of congenital ostium secundum atrial septal defect associated with acquired mitral valve stenosis (usually rheumatic). This unusual cardiac entity is difficult to diagnose clinically because each lesion alters the hemodynamics and clinical characteristics of the other. The resulting clinical manifestations depend chiefly on the size of the defect, the severity of the mitral stenosis, and the compliance of the right ventricle. We present a classic case of Lutembacher's syndrome and illustrate the pitfalls and advantages of echocardiography in the correct diagnosis of the syndrome.