Aim: The aim if this study was to present our experience in the operative management of diseased thoracic aorta in patients with Marfan Syndrome (MFS). In the period 1993-2015 86 patients with MFS and diseased thoracic aorta were treated in our center. Methods: An open surgical treatment was performed in 63 cases and an endovascular exclusion was performed in 23 cases (including hybrid treatments). The larger part of those interventions were elective, and 21 cases were in urgent fashion. Result:. The perioperative mortality was 4.8%. At 30 postoperative day paraplegia was observed in 8.1 % of patients, renal and respiratory failure in 5.8% and 8.1%, respectively. After a mean follow-up of 13.2+3.6 years long-term survival rate was 79.1%. In the group of patients treated with open surgery we observed three cases of Carrell's patch aneurysm, which required open reintervention. In the endovascular cases we observed four cases of graft migration, two cases of aortic antegrade dissection and five cases of endoleak with significative sac growth. Conclusion: Perioperative mortality and morbidity after open and endovascular repair were acceptable in this series in patients with MFS. Because of the high risk of complications and reinterventions after TEVAR, however, we currently limited aortic stent grafting in selected patient in whom an aortic open repair is contraindicated or as bridge technique in urgent fashion.

Thoracoabdominal aortic aneurysms in Marfan patients

KAHLBERG, ANDREA LUITZ;TSHOMBA, YAMUME;MELISSANO , GERMANO;CHIESA, ROBERTO
2015-01-01

Abstract

Aim: The aim if this study was to present our experience in the operative management of diseased thoracic aorta in patients with Marfan Syndrome (MFS). In the period 1993-2015 86 patients with MFS and diseased thoracic aorta were treated in our center. Methods: An open surgical treatment was performed in 63 cases and an endovascular exclusion was performed in 23 cases (including hybrid treatments). The larger part of those interventions were elective, and 21 cases were in urgent fashion. Result:. The perioperative mortality was 4.8%. At 30 postoperative day paraplegia was observed in 8.1 % of patients, renal and respiratory failure in 5.8% and 8.1%, respectively. After a mean follow-up of 13.2+3.6 years long-term survival rate was 79.1%. In the group of patients treated with open surgery we observed three cases of Carrell's patch aneurysm, which required open reintervention. In the endovascular cases we observed four cases of graft migration, two cases of aortic antegrade dissection and five cases of endoleak with significative sac growth. Conclusion: Perioperative mortality and morbidity after open and endovascular repair were acceptable in this series in patients with MFS. Because of the high risk of complications and reinterventions after TEVAR, however, we currently limited aortic stent grafting in selected patient in whom an aortic open repair is contraindicated or as bridge technique in urgent fashion.
2015
Aortic aneurysm; Endovascular procedures; Marfan Syndrome; Thoracic; Surgery; Cardiology and Cardiovascular Medicine
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11768/9102
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