Doppler echocardiographic confirmation of recurrent atrial septal defect stenosis in infants with mitral valve atresia

Abstract Children with complex congenital heart defects, including mitral valve atresia and ventricular septal defect, require free egress of blood from the left atrium through a nonrestrictive atrial septal defect (ASD). If an adequate naturally occurring ASD is not present at birth, balloon atrial septostomy may be required during the newborn period. Although the ASD so produced may initially decompress the left atrium, progressive stenosis may subsequently occur, producing left atrial hypertension and clinical signs and symptoms of pulmonary venous obstruction. Such progressive obstruction appears to characterize the natural history of these lesions. 1–3 We report 3 cases in which restenosis of an ASD occurred in patients with mitral valve atresia. In each case the stenotic ASD was demonstrated by 2-D echocardiography. Presence of a high-velocity jet from the left to the right atrium was detected by pulsed or continuous-wave Doppler echocardiography at the region of the visualized stenotic ASD. Quantitation of the interatrial gradient by Doppler was validated by simultaneous catheterization in 1 case. In the other 2 cases catheterization atrial pressures could not be determined, and atrial obstruction was confirmed at operation. Doppler echocardiography is a valuable additional noninvasive technique for diagnosis and quantitation of restrictive ASD in patients with mitral valve atresia.