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  • Title: [Fontan procedure for asplenia syndrome].
    Author: Miyaji K.
    Journal: Kyobu Geka; 2003 Apr; 56(4):304-7. PubMed ID: 12701194.
    Abstract:
    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.
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