Pulmonary arteriovenous malformation is a rare disease leading to cyanosis, where there is a direct relation between the pulmonary artery and pulmonary vein without a capillary structure. Arteriovenous fistulae may be single or multiple. Clinical signs emerge depending on the size of the fistulae and amount of shunt. Due to the advancements in transcatheter devices and increased experience render enable the fistula embolisation procedure as an alternative to surgical treatment. Extracorporeal membrane oxygenation is used to support the patient haemodynamically and respirationally in cases of treatment-resistant, severe and revocable cardiac or pulmonary sufficiency. This paper presents an infant patient with pulmonary arteriovenous malformation, who had haemodynamic instability due to severe hypoxia and received successful transcatheter fistula embolisation via extracorporeal membrane oxygenation under emergency conditions.

The development of pulmonary atteriovenous fistulas after bidirectional cavopuomonary operat-tions, such as the bidirectional Glenn shunt and Kawashima's procedure, has raised concern. Development of these fistulas, which are more frequent than initially thought, can represnt a limiting factor in the late outcome of these patients and may even limit the indication for these of surgery. Whether the fistulas can be reversed by transforming the surgical procedures has yet to be established. In the hope of avoiding this kind of complication, thought to be caused by the lack of passage of a hypothetical hepatic factor through the pulmonary circulation. wedevelped an inverred type of gidirectional cavopulmonary connection in which the blood coming from the liver perfuses immediately both lungs. This is made possi-ble by shuntiong via an intra-atrial tunnel the blood from the superior caval vein directly to the left atrium, and the blood from the inferior caval vein to the right branch of the pulmonary trunk (keeping its bifurcation intact). We describe findings in two patients undergoing successful surgery with this technique. Serial follow -up with cohtrast echocardiography did not show evidence of arteriovenous pulmonary fistulas.Despite our numbers being small, and the time of follow-up being limited, we believe that it is importantto document these and similar cases

A 37-year-old man with tricuspid atresia, in whom a chronic right apical aspergilloma had stimulated formation of extensive aortopulmonary collateral circulation, suffered reversal of flow within his long-standing fistulous Glenn anastomosis. By reversing the flow through the pulmonary arteriovenous fistula and raising oxygen saturation in the right atrium, the acquired aortopulmonary collateral circulation prevented the increase in cyanosis which usually occurs when fistulous changes develop late after the Glenn operation.

Abstract Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.