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

Abstract

To determine the late incidence of pulmonary arteriovenous malformations after bidirectional Glenn in patients with azygos continuation of the inferior vena cava (Kawashima operation). From 1990 to 2006, 21 patients underwent a Kawashima procedure at a median age of 2.3 years (0.5-8 years). Underlying anatomy included atrioventricular septal defect [14], double-outlet right ventricle [13], pulmonary atresia [3], pulmonary stenosis [11], total anomalous pulmonary venous drainage [4] and bilateral superior vena cavae [14]. There was one hospital death after Kawashima due to low output syndrome. Follow-up was complete in all but one patient. Two patients died 23 days and 4 years after Kawashima following cardiac reinterventions (one atrioventricular valve replacement and one Fontan completion). Thirteen patients developed pulmonary arteriovenous malformations after a median of 4 years (2-9 years) after Kawashima. Freedom from development of arteriovenous malformations was 47% at 5 years (95% CI: 23-69%). A total of 16 patients underwent Fontan completion, 12 for cyanosis related to pulmonary arteriovenous malformations and four for decreased exercise capacity. Only three patients were left without Fontan completion at 4, 9 and 13 years after Kawashima. The two patients who had more than 9 years of follow-up after Kawashima had antegrade flow preserved between the ventricle and the pulmonary arteries. 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.