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Surgical Treatment of Siblings With Marfan Syndrome

Kaan Kirali, MD, Necmettin Yakut, MD, Mustafa Güler, MD, Denyan Mansurolu, MD, Suat Ömerolu, MD, Esat Akinci, MD, Ali Gürbüz, MD, Cevat Yakut, MD

Department of Cardiovascular Surgery Kouyolu Heart and Research Hospital Istanbul, Turkey

For reprint information contact: Kaan Kirali, MD Tel: 90 216 325 5457 Fax: 90 216 339 0441 email: kosuyolu{at}superonline.com Department of Cardiovascular Surgery, Kouyolu Heart and Research Hospital, Kadiköy, Istanbul 81020, Turkey.

Rupture of an ascending aortic aneurysm is the most common cause of death in patients with Marfan syndrome, while type-A aortic dissection is the second most common fatal lesion. The aortic root dimension and the rate at which it increases are the best indications for surgical treatment. We regard aortic enlargement to 5.5 cm as the upper limit of safety and we are inclined to advocate surgery at an earlier stage in high-risk families. We report our results of the surgical management of aortic aneurysm in the two siblings; a third sibling is currently being followed medically.