Abstract
3/2009
vol. 6
DZIELIMY SIĘ DOŚWIADCZENIEM
Mitral insufficiency and aortic arch aneurysm in a woman with Marfan syndrome treated twice in the cardiac surgery unit
Kardiochirurgia i Torakochirurgia Polska 2009; 6 (3): 289–292
Online publish date: 2009/09/23
Marfan syndrome is an autosomal dominant disorder of the connective tissue. Mutation in the fibrillin gene locus is believed to be responsible for impaired structural integrity of the elastin network. Symptoms of Marfan syndrome involve the skeletal, ocular and cardiovascular systems.
We report a case of a 26-year-old woman with Marfan syndrome referred for surgical treatment because of mitral insufficiency and aortic aneurysm. The patient was operated on twice. As a 12-year-old girl she underwent aortic root replacement with composite graft (Bentall-de Bono procedure). After 7 years she was reoperated on. The third operation involved mitral valve replacement and resection of the ascending aorta and the arch due to a large aneurysm located distally to the previous anastomosis. The cardiopulmonary bypass was initiated through the innominate artery. A few weeks after the operation the patient required vacuum-assisted therapy due to sternal wound infection.
We report a case of a 26-year-old woman with Marfan syndrome referred for surgical treatment because of mitral insufficiency and aortic aneurysm. The patient was operated on twice. As a 12-year-old girl she underwent aortic root replacement with composite graft (Bentall-de Bono procedure). After 7 years she was reoperated on. The third operation involved mitral valve replacement and resection of the ascending aorta and the arch due to a large aneurysm located distally to the previous anastomosis. The cardiopulmonary bypass was initiated through the innominate artery. A few weeks after the operation the patient required vacuum-assisted therapy due to sternal wound infection.
Keywords
Marfan syndrome, aortic aneurysm
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