Hs. Hsu et al., RIGHT-TO-LEFT INTERATRIAL COMMUNICATIONS AFTER THE MODIFIED FONTAN PROCEDURE - IDENTIFICATION AND MANAGEMENT WITH TRANSCATHETER OCCLUSION, British Heart Journal, 74(5), 1995, pp. 548-552
Objective-To describe unusual venous communications from the right to
the left atrium resulting in cyanosis after the modified Fontan proced
ure, and their management with transcatheter occlusion. Methods-Betwee
n September 1999 and November 1994, eight patients were assessed for p
ersistent cyanosis after a modified Fontan procedure. Desaturation was
found to be caused by unusual venous shunts originating at atrial lev
el, and transcatheter occlusion with either a double umbrella or coil
was attempted. Results-Three types of venous channels were identified.
The first type of communication consisted of thin long tortuous chann
els originating from the right atrial wall, and draining into the left
atrium through a capillary network. The second type of communication
was in the superior anterior portion of the atrial baffle, incorporati
ng the pectinate muscles of the right atrium, draining into the neolef
t atrium. These channels were shorter and often fanned out into small
vessels toward the right atrial appendage. In each instance, the shunt
s were in the superior suture Line of a lateral tunnel modification of
the Fontan procedure. The third type of communication originated from
the inferior vena cava, connecting inferior phrenic veins to pericard
ial veins and subsequently to the left atrium, at or close to the osti
um of the left pulmonary veins. Before device occlusion, the room air
aortic oxygen saturation was 88(SD 4)% (range 84% to 94%), and increas
ed to 95(3)% (range 91% to 100%) following occlusion (PL much less tha
n 0.001). The mean right atrial pressure was 14(4)mm Hg and remained u
nchanged after occlusion. In six patients there was complete shunt obl
iteration, while in two both occluded with umbrella devices, a small r
esidual leak persisted. No complication occurred during or immediately
after catheterisation. Conclusions-Unusual venous communications can
evolve after the Fontan procedure, resulting in the development or per
sistence of cyanosis. Some of these communications may be present preo
peratively as normal veins draining into the right atrium, enlarging w
ith the increased atrial pressure after surgery. These observations af
fect long term function after the Fontan procedure. Transcatheter occl
usion of these communications is technically feasible and effective, a
lthough recurrence may occur.