Occlusion of Pulmonary Arteriovenous Malformations Using the Amplatzer Vascular Plug/Duct Occluder for a Family With Hereditary Hemorrhagic Telangiectasia

Abstract

An 8-year-old girl was referred because of frequent epistaxis and acrocyanosis. The family history showed that her father, aunt, uncle, and grandfather also experienced recurrent epistaxis. Based on her clinical information, we diagnosed her condition as hereditary hemorrhagic telangiectasia, also known as Rendu-Osler-Weber disease, which is characterized by multiple telangiectases, recurrent epistaxis, arteriovenous malformation, and familial inheritance. Chest radiography and computed tomography (CT) showed two large pulmonary arteriovenous fistulas (PAVFs) in the posterobasal and mediobasal segment of the left lower lung (Fig. 1). Percutaneous transcatheter embolization then was planned. After right heart catheterization and pulmonary angiography had been performed, we confirmed a large PAVF (diameter, 8.2 mm) in the posterobasal part of left lung, with a smaller lesion (diameter, 2.9 mm) in the pulmonary artery of the left middle lobe. A 10 9 12-mm Amplatzer duct occluder (AGA Medical Corporation, Golden Valley, MN, USA) was selected for the larger PAVF, and a Gianturco coil (4 9 3 mm; Cook Inc., Bloomington, IN, USA) was chosen for the smaller feeding artery (Fig. 1). After a successful procedure, percutaneous oxygen saturation at rest rose to more than 95% with room air compared with about 71% before the procedure. Recently, the girl’s father also was evaluated for mucocutaneous telangiectases on his tongue and in his nasal mucosa. Chest radiography and multidetector CT again showed bilateral multiple PAVFs. A cardiac catheterization with pulmonary angiography showed four large PAVFs of the right upper (feeding vessels of 2.8 mm), right lower (3.5 mm), left upper (3.2 mm), and left lower (4.2 mm) lung. Considering their large sizes, we decided to perform percutaneous closure using Amplatzer vascular plugs for all four PAVFs. Devices 30–50% larger than the selected vessel diameter were chosen: 4 9 7 mm for the right upper lesion, 6 9 7 mm for the right lower and left upper lesion, and 8 9 7 mm for the left lower lesion. Angiograms obtained at the end of each procedure confirmed good positioning of the devices with no significant residual flow (Fig. 2). About 20–30% of patients with hereditary hemorrhagic telangiectasia are thought to be affected with PAVFs [4]. Various methods for transcatheter embolization have been reported, including coils and detachable balloons. Although transcatheter embolization with coils is considered the preferred method for PAVFs, it is not ideal for large fistulas with large feeding arteries due to disadvantages such as the risk for paradoxical device embolization, the need for multiple coils, and the high recanalization rates [1–3]. The Amplatzer vascular plug/duct occluder has been reported as a new alternative for the occlusion of PAVFs. An additional advantage of the Amplatzer vascular plug is the relatively smaller introduction system than that used for the Amplatzer duct occluder. Although most reported cases involve adult patients, the reported familial case including an 8-year-old girl demonstrates that the Amplatzer vascular plug and duct occluder also provide excellent results for young patients with multiple large PAVFs. M. J. Cho Department of Pediatrics, Pusan National University Hospital, Ami-dong, Seo-gu, Busan 602-739, Republic of Korea