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.