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Ann Thorac Surg 2005;80:1604-1609
© 2005 The Society of Thoracic Surgeons

Original article: Cardiovascular

Creation of a Brachial Arteriovenous Fistula for Treatment of Pulmonary Arteriovenous Malformations After Cavopulmonary Anastomosis

Doff B. McElhinney, MD a , c , * , Audrey C. Marshall, MD a , c , Peter Lang, MD a , c , James E. Lock, MD a , c , John E. Mayer, Jr, MD b , d

a Department of Cardiology, Children's Hospital, Boston, Massachusetts
b Department of Cardiac Surgery, Children's Hospital, Boston, Massachusetts
c Department of Pediatrics, Harvard Medical School, Boston, Massachusetts
d Department of Surgery, Harvard Medical School, Boston, Massachusetts

Accepted for publication May 9, 2005.

* Address correspondence to Dr McElhinney, Department of Cardiology, Children's Hospital, 300 Longwood Ave, Boston, MA 02115 (Email: doff.mcelhinney{at}cardio.chboston.org).

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) occur in approximately 20% of patients after unidirectional superior cavopulmonary anastomosis (CPA), and frequently after bidirectional CPA in patients with polysplenia syndrome. It is hypothesized that exclusion of a growth-modulating factor produced in the liver may predispose to PAVM formation. Resolution of PAVMs after inclusion of hepatic venous effluent into the cavopulmonary circulation has been reported. An upper extremity systemic arteriovenous (AV) fistula may be created to augment pulmonary blood flow and improve oxygenation in hypoxemic patients with CPA, but there has been no systematic investigation of the effects of such fistulas on PAVMs after CPA.

METHODS: We studied 11 patients with PAVMs who underwent creation of a brachial AV fistula a median of 11 years after CPA.

RESULTS: Eight patients had discontinuous pulmonary arteries or unilateral flow of a bidirectional CPA and were not considered good candidates for Fontan completion; the other 3 patients had polysplenia and unilateral hepatic venous streaming after Fontan completion. Three patients died of progressive complications of their heart disease 4 to 18 months after AV fistula creation. Pulmonary arteriovenous malformations resolved after creation of a brachial AV fistula in 4 of 5 surviving patients with unilateral flow of a superior CPA, but in none of 3 patients with polysplenia who had unilateral hepatic venous streaming after Fontan completion and PAVMs in the contralateral lung.

CONCLUSIONS: These findings are consistent with the "hepatic factor" hypothesis, according to which the development of PAVMs is facilitated when an unidentified factor produced or metabolized in the liver does not reach the pulmonary circulation before traversing another capillary bed. Patients with unilateral superior CPA flow and PAVMs who are not considered candidates for Fontan completion may benefit from a brachial AV fistula.


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Incorporation of the Hepatic Veins Into the Cavopulmonary Circulation in Patients With Heterotaxy and Pulmonary Arteriovenous Malformations After a Kawashima Procedure
Doff B. McElhinney, Jacqueline Kreutzer, Peter Lang, John E. Mayer, Jr, Pedro J. del Nido, and James E. Lock
Ann. Thorac. Surg. 2005 80: 1597-1603. [Abstract] [Full Text] [PDF]



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