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Treatment of a large PAVF in a child after TCPC

Introduction: Late complications of RV by-pass procedures appear as arrhythmias, protein losing enteropathy, chronic heart failure and PAVF. Treatment of large PAVF is particularly difficult and may require complete resection of pulmonary segments affected by a fistula. Case study: A girl P.T, born 08/2000 with a CHD, with basic diagnoses: Situs inversus abdominalis, left atrial isomerism, levocardia, functional single ventricle, single AV-valve, TGA, right aortic arch, infundibular and valvular PS, LVCS, azygos continuity, RVCS. Treatment: Ao-PA central shunt (Zagreb, 10/00), bidirectional Glenn (Zg, 04/01), TCPC with fenestration (Zg, 12/02). During the first year the child had no difficulties, then a deeper cyanosis started developing which warned of PAVF development. Selective angiocardiography established large PAVF in the medium and lower lobe of the right lung. Test-occlusion by transcatheter closing of pulmonary branches of the right lung in the medium and lower lobe and closing the fenestra with an Amplatzer plug increased saturation from 60% to 90% After the test, the Amplatzer plug was implanted, 12 mm on the fenestration and 10 mm on the fistulas (01/07 DHM). Nonetheless, deep cyanosis was developing due to new R-L shunt development on the intrapulmonary fistulas level (R-L shunt 59%). Resection of the medium and lower lobe of the right lung was performed. 18 months after the surgery she is still saturated >90%. Conclusion: Large pulmonary arteriovenous fistulas sometimes can not be successfully closed using interventional diagnostics procedure and should be treated with pulmectomy of the pulmonary lobes affected by the fistulas.

pulmonary arteriovenous fistula; TCPC

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