Congenital absence of the pulmonary valve associated with ventricular septal defect.
The authors describe 5 cases of an unusual variant of tetralogy of Fallot with a characteristic clinical course. Its most prominent clinical manifestations are recurrent and severe respiratory infections caused by compression of the inferior trachea and bronchi by a dilated pulmonary artery. Two of the patients died of irreversible respiratory insufficiency. Of the 3 surviving patients one is doing comparatively well.
D004562:Electrocardiography; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007223:Infant; D011664:Pulmonary Valve / Q000002:abnormalities*; D013771:Tetralogy of Fallot / Q000175:diagnosis