[Fontan procedure for asplenia syndrome].
BACKGROUND
Historically the Fontan operation in patients with single ventricle asplenia syndrome has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome.


METHODS
A retrospective review of 11 patients with asplenia syndrome who underwent a Fontan operation between 1996 and 2002 was performed.


RESULTS
Anomalies of pulmonary venous return included 5 patients (46%) and 4 patients with an isolated hepatic venous return from inferior vena cava (36%). The type of Fontan procedure included 6 patients with an extracardiac tube-graft, 3 with a lateral tunnel modification, 1 with an intra-atrial tube graft, and 1 with an intra-extra atrial tube graft. A fenestration was placed in 3 patients (27%). There were no early deaths and 2 late hospital deaths (18%) due to tachyarrythmia and failed Fontan circulation. There were no late deaths and no complications during follow-up periods (mean 3 years). Of 21 patients diagnosed with asplenia syndrome between 1990 and 2000, overall survival was 57% and 12 patients underwent a Fontan-type operation.


CONCLUSIONS
The Fontan operation can now be performed in patients with asplenia syndrome with good survival. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a fenestration contribute to improved outcome.
Miyaji
Kagami
K
Department of Cardiovascular Surgery, Kanagawa Children's Medical Center, Yokohama, Japan.
D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000379:methods; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013154:Spleen / Q000002:abnormalities* / Q000601:surgery*