ABSTRACT
A 62-year-old man with Marfan syndrome had a modified Bentall procedure and total arch replacement for annuloaortic ectasia, aortic insufficiency and thoracic aortic aneurysm fifteen years ago at another hospital. A follow-up CT revealed bilateral coronary artery aneurysms and an aortic root pseudoaneurysm, and thus he was referred to our hospital. The previous prosthetic valve was removed, followed by the re-Bentall procedure. Coronary artery aneurysms were resected and consequently coronary arteries were reconstructed directly. Although the shortcoming of the Bentall procedure was pseudoaneurysm, the outcomes of the modified Bentall procedure have shown some improvements. However, as there is still a high risk of postoperative complication in connective tissue diseases, long-term follow-up is required.