Congenital Anomalies of the Mitral Valve

The mitral valve is defined as the inlet valve to the anatomic left ventricle, regardless of ventriculoarterial concordance. Congenital mitral valve disease frequently occurs in combination with coarctation or hypoplasia of other left-sided structures, including the left ventricle, aortic valve, and aortic arch. Surgical management of mitral valve disease in children began in the 1920s with treatment of rheumatic mitral stenosis. The normal mitral valve can be subdivided into the annulus, leaflets, chordae tendineae, and papillary muscles. Etiology of mitral valve dysfunction includes congenital malformations and acquired disease. Both mitral stenosis and regurgitation lead to left atrial and pulmonary venous hypertension in the presence of intact atrial septum due to compensation for increased pressure and volume loads. Radiographic evidence for left atrial hypertension includes the presence of cardiomegaly with left atrial enlargement and prominent interstitial lung markings with increased pulmonary vascularity.