Intermittent acute profound cyanosis in patients with EA-TEF is usually
due to near complete collapse of the trachea. However, the possibility
of an anatomical shunt has to be ruled out because congenital heart
malformations associated with right-to-left shunts (such as valve
atresia, Tetralogy of Fallot, anomalous pulmonary venous return, and
septal defects or patent ductus arteriosus) are present in more than one
quarter of the patients80,84 and they are independent
predictors of survival125. For children with no
history of congenital heart disease an echocardiogram is useful in order
to investigate for presence of vascular abnormalities such as
right-sided or double aortic arch, and aberrant right or left subclavian
arteries that may complicate the clinical presentation by causing or
exacerbating the degree of
tracheomalacia37,78,80,81,126. Definitive confirmation
will require a CT-angiogram or an MRI/MRA or cardiac catheterization.