title: Masked subaortic stenosis in ostium primum atrial septal defect: recognition and treatment.
abstract: Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic "goose-neck" deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.
mesh_terms: D000293:Adolescent; D001024:Aortic Valve Stenosis / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D006344:Heart Septal Defects, Atrial / Q000150:complications* / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D007223:Infant; D008943:Mitral Valve / Q000002:abnormalities