Should we always plan a Fontan completion after a Kawashima procedure?

Abstract

Unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure will ultimately develop pulmonary arteriovenous malformations. Elective non-fenestrated Fontan completion in the years following Kawashima procedure should be recommended.

Role of cardiac MRI and CT in Fontan circulation

“…2) [5]. The distinctive risk of developing pulmonary arteriovenous malformations (PAVM) post Kawashima has led to the re-direction of the hepatic veins to the pulmonary arteries thus completing a Fontan and subsequent reduction in PAVM [6].…”

A retrospective analysis of cases with left atrial isomerism

“…[20] Yapılan çalışmalarda Kawashima ameliyatı sonrası erken dönemde hepatik venlerin pulmoner artere yön-lendirilmesinin pulmoner arteriyovenöz fistül gelişimini önlediği, progresif siyanozu azaltarak mortalite ve morbititeye pozitif katkı sağladığı gösterilmiştir. [11,21] Bizim çalışmamızda da Kawashima ameliyatı sonrası izlemde progresif siyanoz gelişen altı olguya ekstrakardiyak kondüitle hepatik ven yönlendirilmesi yapılmak zorunda kalındı. Literatür verileri ve gözlemlerimiz ışığında, 2012 yılından itibaren toplam dört olguda aynı seansta hepatik venleri de pulmoner dolaşıma dahil ettik.…”

Cross-sectional imaging of the Fontan circuit in adult congenital heart disease

“…First performed by Kawashima in 1984, this procedure comprises division of the SVC distal to the drainage of the azygos and anastomosis of the cranial aspect of the SVC to the pulmonary artery. 8,9 Bilateral SVCs may be seen in association with an interrupted IVC; in this instance, a bilateral bidirectional Glenn anastomosis is often fashioned. The result post-Kawashima procedure is a near total cavopulmonary connection (due to systemic venous return to the SVC via azygos continuation).…”

“…This important distinction has been linked with an increased risk of pulmonary arteriovenous malformations post-Kawashima procedure. 9 The incremental development of surgery over time means that the different types of Fontan circuit are all still encountered in adult congenital heart disease patients. Although the anatomy can vary considerably, the principle of systemic venous drainage direct to the pulmonary arteries and a single ventricle supplying the systemic artery prevails.…”