Pulmonary sequestration presenting as mitral valve insufficiency.

Although pulmonary sequestrations commonly present with infectious complications, problems relating to high blood flow through the lesion are rarely apparent. A 4-year-old girl was referred for cardiac catheterization and evaluation for mitral valve surgery. An echocardiogram had demonstrated left atrial and ventricular enlargement and significant mitral regurgitation with an enlarged valve annulus. Angiography results showed a very large aorta to left atrial shunt through an unsuspected intralobar sequestration. Lobectomy with removal of the sequestration resulted in significant improvement in cardiac chamber size and function over a 2.5-year follow-up period, thus obviating the need for cardiac surgery and removing a potential source of infection. Careful evaluation of chest imaging studies will lead to the correct diagnosis and treatment in patients with pulmonary sequestration who are thought initially to have primary cardiac disease.