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Novel Technique for Staged Cavopulmonary Connection in Patients with Interrupted Inferior Vena Cava and Azygos Continuation
J. Nagendran, D. H. Freed, S. Aboelnazar, I. M. Rebeyka*. University of Alberta, Edmonton, AB, Canada,
BACKGROUND:
The bidirectional cavopulmonary anastomosis (BCPA) is an operation that increases effective pulmonary blood flow and reduces the volume load on the ventricle, and is the intermediate stage before completion to a Fontan circulation in patients with single ventricle anatomy. The BCPA creates an end-to-side anastomosis between the superior vena cava (SVC) to the superior aspect of the right pulmonary artery (R-PA). The standard method of creating a BCPA in patients with an interrupted inferior vena cava (IIVC) and azygos continuation (the Kawashima operation) is complicated by development of pulmonary arterio-venous fistulae. Additionally, the Kawashima operation must be delayed (> 18 months old) as it creates a Fontan-type circulation with exclusion of hepatic venous drainage. Currently, there are no alternative options for a BCPA in patients < 10 months old with IIVC and azygos continuation. We describe for the first time a novel method for creation of a BCPA in the pathology of IIVC with azygos continuation. This alternate operation produces typical BCPA physiology in patients < 10 months old as an intermediate stage to the completion Fontan.
METHODS:
In this alternate procedure, the SVC is transected distal to the azygos vein and the cardiac end is over sewn, thus allowing systemic venous return from the azygos vein to drain into the proximal SVC and into the atrium. An anastomosis is created between the transected end of the SVC and the right-pulmonary artery (Figure). A retrospective chart review was completed for all patients with IIVC who underwent the alternate procedure. Peri-operative course and long-term outcomes, including completion Fontan operations were analyzed.
RESULTS:
Four patients with IIVC underwent the alternate procedure with a mean age of 7.2 +/- 1.8 months. All patients survived and the mean change in post-operative arterial oxygen saturation was 2.0 +/- 7.4%. None of the patients developed evidence of pulmonary arterio-venous fistulae at any time in follow-up. A completion Fontan operation was performed at mean age of 37.2 +/- 5.1 months.
CONCLUSIONS:
The novel procedure allows creation of a standard BCPA in patients with IIVC and azygos continuation, which can be performed much earlier in life than the Kawashima operation. Conversion to a completion Fontan can be done at the usual age for patients being palliated with a Fontan-type circulation. This facilitates management of these complex patients in a more routine fashion as with other congenital heart diseases with single ventricle anatomy. 
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