%0 Journal Article
%T Penetration of left and right atrial wall and aortic root by an Amplatzer atrial septal occluder in a nine year old boy with Marfan syndrome: Case report
%A Florian Loeffelbein
%A Christian Schlensak
%A Sven Dittrich
%J Journal of Cardiothoracic Surgery
%D 2008
%I BioMed Central
%R 10.1186/1749-8090-3-25
%X A nine-year-old boy with Marfan syndrome and a 22 mm atrial septal defect (ASD) was treated successfully by interventional closure of his ASD by placing a 24 mm Amplatzer septal occluder. Follow up examinations showed a good result but an increasing enlargement of aortic root, so the patient was scheduled for operation. Intraoperative findings showed a perforation of the left atrial roof and the non-coronary sinus by penetration of the occluder device as well as penetration into the right atrial wall. The occluder was resected, the ASD was closed and the aortic sinus was reconstructed using a Dacron patch.We describe the first case of a patient with Marfan syndrome and an interventional closure of an ASD. Due to alterations of the connective tissue, as it is typical for patients with Marfan syndrome, the Amplatzer occluder probably perforated adjacent structures more easily as in non-affected individuals. Amplatzer occluders should be used with caution and follow up examinations should be performed in short intervals.Marfan syndrome is a relatively common genetic disorder with an estimated prevalence of 1: 3,000 to 1:10,000. It is most often caused by mutations in the FBN1 (Fibrillin) gene [1,2]. Patients typically present with signs and symptoms related to alterations of their connective tissue including tall stature, recurrent back pain, pectus excavatum, dural ectasia, subluxation of the lens, aneurysm of the aortic root with subsequent aortic regurgitation, and aneurysms of the ascending, descending, or thoracic aorta which can result in rupture and death.Marfan syndrome is an autosomal dominant disorder with the onset of symptoms occurring in the first decade of life. Nearly 50 percent of patients have to undergo aortic surgery in their lifetime resulting in reconstruction or replacement of the aortic root or total of this vessel's parts [3]. In addition, the occurrence of septal defects in patients with Marfan syndrome is not common.We report a nine-year-old b
%U http://www.cardiothoracicsurgery.org/content/3/1/25