Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

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.

Loeffelbein F, Schlensak C, Dittrich S

Clinic of Pediatric Cardiology, Children's University Hospital, Freiburg, Mathildenstrasse 1, D-79106 Freiburg, Germany. florian.loeffelbein@uniklinik-freiburg.de.

ABSTRACT: BACKGROUND: To describe complications associated with Amplatzer septal occluders in a patient with Marfan syndrome CASE PRESENTATION: 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. CONCLUSION: 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.

Published 29 May 2008 in J Cardiothorac Surg, 3: 25.
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