Osler-Rendu-Weber syndrome: congenital arteriovenous intrapulmonary fistula treated using a percutaneous Amplatzer plug

V. Conti1, F. Fiorucci1, M. Serpilli1, A. Patrizi1, G. Paone1,4, P. Neri1, G. Giannunzio1, M. Guzzo Pirozzo3, C. Fiorucci2, G. Lucantoni1

1 C.U.B.E. Dipartimento di Scienze Cardiovascolari e Respiratorie, Università di Roma “La Sapienza”, Ospedale “Carlo Forlanini”, Rome (Italy) 2 UOC di Radiologia, Ospedale di Bracciano, Rome (Italy) 3 UOC di Medicina Generale, Fondazione Poliambulanza, Brescia (Italy) 4 Fondazione Don Gnocchi, “Santa Maria della Pace”, Rome (Italy)

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• Respiratory Medicine

Pulmonary arteriovenous fistulas (PAVFs) are rare vascular malformations (PAVMs) of the lung that could lead to severe hypoxiemia due to right-to-left intrapulmonary shunts. They may occur as isolated entities or associated with Osler-Rendu-Weber syndrome or hereditary haemorrhagic telangiectasia (HHT).
We report a case of a 70 years old woman with Rendu-Osler-Weber disease and a large arteriovenous malformation involving the left pulmonary artery.
We describe the successful transcatheter occlusion of the PAVF using an Amplatzer® vascular plug. This work is an attempt to focuse the attention on pulmonary arteriovenous malformations and on percutaneous treatment as an alternative to surgery, that consists of a conservative lung resection.

Corresponding Author: Fabio Fiorucci, MD; e-mail: fabiofiorucci@tiscali.it

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