We describe a three-year-old boy who presented with recurrent expectoration
of bronchial casts six months following creation of a fenestrated lateral
tunnel Fontan circulation for pulmonary atresia with intact ventricular sep
tum. Cardiac catheterization demonstrated elevated central venous pressure
with two areas of stenosis within the Fontan circuit, specifically at the j
unction of the right superior caval vein and the right pulmonary artery, an
d between the atrial baffle and the right superior caval vein. Insertion of
Palmaz stents in these areas resulted in a reduction in central venous pre
ssure, and a transient reduction in production of casts. Eight weeks after
catheterization, however, he produced further casts, which resulted in hypo
xia, respiratory arrest and death. We reviewed the autopsied specimens obta
ined from patients with the Fontan circulation over an eleven-year period a
t our institution in order to ascertain the prevalence of subclinical produ
ction of bronchial casts. We found no casts in the thirteen patients examin
ed. Hemodynamic assessment is vital in all patients who develop this syndro
me, and should be the primary focus of investigation, rather than solely di
recting efforts at lysis of casts.