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The Journal of Thoracic and Cardiovascular Surgery, Vol 96, 212-218, Copyright © 1988 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
RA Humes, RH Feldt, CJ Porter, PR Julsrud, FJ Puga and GK Danielson
From 1975 through 1986, 49 patients with asplenia (23 patients) or
polysplenia (26 patients) syndromes underwent a modified Fontan operation.
All patients had anomalous systemic venous return, and 43 (88%) had
anomalous pulmonary venous return. The atrioventricular valve anatomy
varied: 36 patients had a common atrial chamber and common atrioventricular
valve, eight had mitral valve atresia, and five had severe mitral valve
hypoplasia. Redirection of systemic venous return was accomplished by (1)
atrial baffle (29 patients), (2) intraatrial conduit (19 patients), and (3)
extraatrial conduit (one patient). There were 21 (43%) hospital deaths
overall. However, since 1985, six of 22 patients (27%) have died. Increased
mortality was seen in patients requiring atrioventricular valve repair or
replacement (8/11, 73%) and in patients with asplenia (65%). Lower
mortality was seen in patients with polysplenia (24%) and those receiving
an intraatrial conduit (26%), although this decrease also represents the
more recent experience. We conclude: (1) The Fontan operation for patients
with asplenia or polysplenia syndromes has resulted in a significant
(although recently declining) mortality; (2) mortality is higher in
patients with asplenia; (3) patients with atrioventricular valve
insufficiency requiring repair or replacement are at higher risk; and (4)
intraatrial conduits seem to offer promise for successfully accomplishing
this repair.
ARTICLES
The modified Fontan operation for asplenia and polysplenia syndromes
Department of Diagnostic Radiology, Mayo Clinic, Rochester, Minn 55905.
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