Venovenous malformation: a common finding after Kawashima operation

Abstract Objectives: It has been reported that systemic venovenous malformation (VVM) can develop in patients with interrupted inferior vena cava (IVC) and univentricular type of congenital heart disease who undergo superior vena cava to pulmonary artery connection (Kawashima operation). These malformations can lead to profound systemic desaturation postoperatively. However, there have been few reports that characterise the prevalence, anatomic details and clinical correlations of these systemic VVM arising after Kawashima operation. In this study, we describe our experience with VVM after Kawashima operation, and discuss issues regarding their evaluation and postoperative management. Methods: Eight patients with median age 19 months (range: 5-238) who underwent Kawashima operation were subjected to postoperative angiography, prospectively. Sites of VVM origin and entry, as well as their course, were documented. The presence of pulmonary arteriovenous malformations (AVMs) was also documented. Results: At median follow-up of 31 months (range: 16-72 months), a total of 14 VVM were found in different supra- and infra-diaphragmatic sites in six patients (75%); four of them had concomitant pulmonary AVM while the remaining two patients had only pulmonary AVM. Conclusions: Our findings suggest that systemic VVM can occur frequently after Kawashima operation and can produce significant desaturation postoperatively, and hence we support hepatic incorporation. Performing detailed angiographic studies of the supra- and infra-diaphragmatic systemic veins in routine assessment of patients before Kawashima operation is, probably, warranted.