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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 - J Cardiothorac Surg (2008)

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: 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.

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Overhead view of operating field: a: Intraaortic view: 1: venous canula; 2: aortic clamp; 3: inside of aortic wall; asterisk: penetration defect of the non-coronary sinus. b: Intraatrial view: 1: venous canula; 2: aortic clamp; 4: ascending aorta; 5: right atrial appendage; asterisk: septal device and penetration defect of the left atrial roof.
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Figure 2: Overhead view of operating field: a: Intraaortic view: 1: venous canula; 2: aortic clamp; 3: inside of aortic wall; asterisk: penetration defect of the non-coronary sinus. b: Intraatrial view: 1: venous canula; 2: aortic clamp; 4: ascending aorta; 5: right atrial appendage; asterisk: septal device and penetration defect of the left atrial roof.

Mentions: From the surgeon's point of view, the Amplatzer septal device had penetrated from the left atrium into the non-coronary sinus of the aortic root with subsequent aneurysmatic ballooning (Figure 2a and 2b). Also, a covered perforation of the left atrial roof was observed and the right atrial disk of the occluder had penetrated into the roof of the right atrium without perforation. The original atrial septal defect was in a central position with an intact superior rim. In light of these findings, the closure device was removed and the septal defect directly closed. Perforated areas were reconstructed by pericardium and the affected sinus was replaced by a Dacron patch.


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 - J Cardiothorac Surg (2008)

Overhead view of operating field: a: Intraaortic view: 1: venous canula; 2: aortic clamp; 3: inside of aortic wall; asterisk: penetration defect of the non-coronary sinus. b: Intraatrial view: 1: venous canula; 2: aortic clamp; 4: ascending aorta; 5: right atrial appendage; asterisk: septal device and penetration defect of the left atrial roof.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2397405&req=5

Figure 2: Overhead view of operating field: a: Intraaortic view: 1: venous canula; 2: aortic clamp; 3: inside of aortic wall; asterisk: penetration defect of the non-coronary sinus. b: Intraatrial view: 1: venous canula; 2: aortic clamp; 4: ascending aorta; 5: right atrial appendage; asterisk: septal device and penetration defect of the left atrial roof.
Mentions: From the surgeon's point of view, the Amplatzer septal device had penetrated from the left atrium into the non-coronary sinus of the aortic root with subsequent aneurysmatic ballooning (Figure 2a and 2b). Also, a covered perforation of the left atrial roof was observed and the right atrial disk of the occluder had penetrated into the roof of the right atrium without perforation. The original atrial septal defect was in a central position with an intact superior rim. In light of these findings, the closure device was removed and the septal defect directly closed. Perforated areas were reconstructed by pericardium and the affected sinus was replaced by a Dacron patch.

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: 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.

Show MeSH
Related in: MedlinePlus