Failure to thrive, cyanosis, tachycardia, signs
and symptoms of right ventricular failure. Auscultation may reveal fixed,
wide splitting of the second heart sound, a diastolic murmur of tricuspid
origin, and a pulmonary ejection murmur in the presence of pulmonary
hypoplasia. Presentation may be delayed in the presence of a nonrestrictive
atrial septal defect until growth results in the atrial septal defect
becoming restrictive causing a fall in arterial oxygen pressure (PaO2).
The ECG shows right axis deviation and signs of right ventricular strain.
The anomalous vein(s) cause a vertical scimitar shape on chest radiograph.