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Systemic Arteriovenous Fistulae For Endstage Cyanosis Following Cavopulmonary Connection: A Useful Bridge To Transplantation
E. J. Hickey, A. A. Alghamdi, G. S. Van Arsdell, C. A. Caldarone, J. Coles, W. G. Williams*. The Hospital for Sick Children, Toronto, ON, Canada,
BACKGROUND:
Intractable cyanosis after partial- or complete cavopulmonary connection may rarely be managed by creating a systemic arteriovenous fistula (AVF). We investigated the long-term performance of AVF.
METHODS:
All 21 patients who have received AVF at our institution since the 1950s were included in a review. Primary AVF indication was either: 1) suboptimal pulmonary blood flow (N=15) or 2) pulmonary shunting via pulmonary arteriovenous malformations (N=6). AVF failure was defined as absent AVF flow (either via surgical ligation or spontaneous occlusion. Parametric competing risks methodology and univariate risk-hazard analysis were used.
RESULTS:
Overall survival was 73%+/-10 at 15 years from the time of receiving AVF (longest survivor 27.3 years).
All 21 patients had previously undergone second-stage palliation (Glenn shunt, N=13; bidirectional shunt, N=9). Five had undergone Fontan completion.
Death in the presence of a functioning AVF occurred in 5. Patients with bidirectional shunts had a significantly higher risk of death with a functioning AVF in situ (P=.04). High hemoglobin concentrations were associated with best outcome, and levels <170 g/l were associated with disproportionately high risk of death despite a functioning AVF (P<.01).
Failure of AVF occurred in 10 children. Earlier AVF failure was associated with previous Fontan completion (P=.02) and patients who had pulmonary arteriovenous malformations (P=.03).
AVF ligation during cardiac transplantation was the cause of failure in 7 patients. In these 7, the AVF functioned for a median 4.8 years. After transplantation, survival was 67%±19 at 5 years.
CONCLUSIONS:
In patients with high hemoglobin (>170 g/L), AVF may offer an effective bridge (≈5 years) to transplantation in patients where high-risk Fontan is deferred. Their performance is best after uni-directional cavopulmonary connection, and poor after Fontan completion. The ideal indication is inadequate pulmonary blood flow: AVF failure rate is high in the presence of pulmonary arteriovenous malformations (figure). Despite being an end-stage palliative strategy, approximately 75% remain alive 15 years after AVF. 
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