Setyapranata S, Brizard CP, Konstantinov IE, Iyengar A, Cheung M, d’Udekem Y
Eur J Cardiothorac Surg 2011;40:1011-15.
The Kawashima operation has long been considered a definitive palliation in patients with univentricular physiology and interrupted inferior vena cava (IVC) draining into the azygous or the hemiazygous system. This operation consists of a bidirectional superior cavopulmonary anastomosis (BDG) on the side of the drainage of the IVC. This paper from the Royal Children's Hospital, Melbourne, addresses the issue of the late incidence of pulmonary arteriovenous malformations (AVM) in these patients. The development of these malformations has been attributed to the isolation of the lungs from the exposure to hepatic factors.
Between 1990 and 2006, 21 patients underwent a Kawashima procedure at a median age of 2.3 years (0.5-8 years). The underlying anatomy included atrioventricular septal defect (n = 14), double-outlet right ventricle (n = 13), pulmonary atresia (n = 3), pulmonary stenosis (n = 11), total anomalous pulmonary venous drainage (n = 4 ) and bilateral superior vena cavae (n = 14). After the Kawashima operation, three patients remained with restricted forward flow from their systemic ventricle to the pulmonary arteries. There was one hospital death after Kawashima due to low-output syndrome. Before hospital discharge, the 20 Kawashima survivors had a median systemic oxygen saturation of 88.5% (76–94%). Follow-up was complete in all but one patient. Median follow-up was 14.5 years (3.6–19.8 years).
Thirteen patients developed pulmonary AVM after a median of 4 years (2–9 years) after Kawashima operation. These malformations were bilateral in eight patients and unilateral in five patients (three in the left lung). Diagnosis of pulmonary AVM was made at a median age of 6.6 years (2.4–16.3 years) and at a median of 4 years (1.9–8.8 years) after the Kawashima operation. A total of 16 patients underwent Fontan completion, 12 for cyanosis related to pulmonary AVM and four for decreased exercise capacity. Only three patients were left without Fontan completion at 4, 9 and 13 years after Kawashima. One of the 13 patients who developed pulmonary AVM died 4 years after Kawashima operation after an urgent repeated atrioventricular valve replacement for valve thrombosis. The rest of the 12 patients underwent Fontan completion (nine extra-cardiac and three lateral tunnels) at a median of 5.5 years (2.0–17.5 years) after Kawashima and at a median age of 7.5 years (3.0–25.6 years). Four of the 11 Fontan survivors had post-Fontan cardiac catheterisation. Pulmonary AVM were still detected in three patients at 4 months and 2 and 5 years post-Fontan. In two of these patients, hepatic flow was demonstrated to be draining only to one side of the lung and the ipsilateral vascular malformations had regressed, while the malformations in the opposite lung persisted. These two patients underwent Fontan revision. Among the seven patients who did not develop pulmonary arteriovenous malformations after the Kawashima, four underwent Fontan operation at 1.5, 2.2, 4.3 and 5.5 years after Kawashima operation. Freedom from development of AVM was only 47% at 5 years (95% CI: 23–69%). The authors concluded that unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure, will ultimately develop pulmonary AVM, and they recommend elective non-fenestrated Fontan completion in the years following Kawashima procedure.