Objective: Cyanosis in conjunction with atrial septal defect does not necessarily indicate the Eisenmenger syndrome. Exceptionally, the right-to-left shunt may result from tricuspid regurgitant flow and its unusual direction.
Methods: We describe thirteen patients with atrial septal defect and accompanying tricuspid regurgitation. In all of them, the tricuspid regurgitant jet was oriented towards the interatrial septum. This was found on the basis of echocardiographic examination and subsequent frame-by-frame analysis of videotape.
Results: Of thirteen patients, cyanosis was observed in 7 (53%). Among those seven, three had moderate pulmonary hypertension (pulmonary artery systolic pressure (PASP) between 40 and 60 mm Hg). The remaining four patients with marked pulmonary hypertension (PASP > 60 mm Hg) underwent cardiac catheterization, which demonstrated low values of pulmonary artery resistance. Except for the one patient with a history of the cerebral embolic event, all were qualified for cardiac surgery. In the postoperative observation they were uneventful and showed clinical recovery and echocardiographic improvement.
Conclusions: Appreciable arterial desaturation and cyanosis in patients with ASD, regarded as uncomplicated, should be followed by careful investigation for the direction of tricuspid regurgitant flow.