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J Thorac Cardiovasc Surg 2010;139:128-134
© 2010 The American Association for Thoracic Surgery

Congenital Heart Disease

Systemic arteriovenous fistulae for end-stage cyanosis after cavopulmonary connection: A useful bridge to transplantation

Departments of Pediatrics and Surgery, Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada

Received for publication January 22, 2008; revisions received October 20, 2008; accepted for publication November 23, 2008.

^_* Address for reprints: William G. Williams, MD, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5 G 1X8. (Email: bill.williams{at}sickkids.ca).

Objective: Intractable cyanosis after partial or complete cavopulmonary connection may rarely be managed by creating a systemic arteriovenous fistula. We investigated the long-term performance of arteriovenous fistulae.

Methods: All 21 patients who received an arteriovenous fistula at The Hospital for Sick Children since the 1950s were investigated using parametric competing risk techniques. Primary arteriovenous fistula indication was (1) suboptimal pulmonary blood flow (N = 15) or (2) pulmonary shunting via pulmonary arteriovenous malformations (N = 6). Arteriovenous fistula longevity was determined by time to "occlusion" (absence of arteriovenous fistula flow via surgical ligation or spontaneous occlusion).

Results: All 21 patients had previously undergone second-stage palliation (Glenn shunt = 13; bidirectional shunt = 9). Five patients had undergone Fontan completion. Death in the presence of a functioning arteriovenous fistula occurred in 5 patients. Patients with bidirectional shunts had a significantly higher risk of death with a functioning arteriovenous fistula in situ (P = .04). High hemoglobin concentrations were associated with best outcome, and levels less than 170 g/L were associated with a high risk of death despite a functioning arteriovenous fistula (P < .01). Arteriovenous fistula occlusion occurred in 10 patients. Earlier occlusion was associated with previous Fontan completion (P = .02) and pulmonary arteriovenous malformations (P = .03). Surgical ligation during cardiac transplantation was the cause of occlusion in 7 patients. In these 7 patients, the arteriovenous fistula functioned for a median of 4.8 years. After transplantation, survival was 67% ± 19% at 5 years. Overall survival was 73% ± 10% 15 years after receiving an arteriovenous fistula (longest survival, 27.3 years).

Conclusion: In patients with adequate hematocrit, arteriovenous fistula offers an effective bridge to transplantation when a high-risk Fontan procedure is deferred. Performance is best after unidirectional cavopulmonary connection and worse in the presence of pulmonary arteriovenous malformations. Survival is 75% at 15 years, despite being considered end stage.