Leon M. Ptaszek, MD, PhD, Kibeom Kim, BA, Amy E. Spooner, MD, Thomas E

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Marfan Syndrome Is Associated With Recurrent Dissection of the Dissected Aorta Leon M. Ptaszek, MD, PhD, Kibeom Kim, BA, Amy E. Spooner, MD, Thomas E. MacGillivray, MD, Richard P. Cambria, MD, Mark E. Lindsay, MD, PhD, Eric M. Isselbacher, MD The Annals of Thoracic Surgery Volume 99, Issue 5, Pages 1616-1623 (May 2015) DOI: 10.1016/j.athoracsur.2014.12.066 Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 Computed tomography angiography images of the evolving aortic dissection anatomy in the patient from case 1. (A) At age 36, a single dissection flap separating the true (T) and false (F) lumens in a descending aorta that is only mildly dilated. (B) At age 46, an image taken when the patient presented with recurrent symptoms, which now demonstrates 2 dissection flaps that separate the true lumen (T), original false lumen (F1), and second false lumen (F2). (C) At age 47, a similar flap configuration is present but the F2 lumen has expanded, resulting in a large thoracoabdominal aneurysm. (D) One year after surgical repair, an intimal flap persists in the aortic arch. The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Computed tomography angiography in a surface-shaded display (left anterior oblique projection) in the patient from case 1, performed at age 47 years. Evident is the ascending thoracic aortic interposition graft from prior repair of the initial type A aortic dissection. The original false lumen (F1) and second false lumen (F2) are visualized spiraling around the true lumen (T). The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 Axial images and planar reformats (A), (B), (D), and (E) in the left-anterior oblique projection (C) and (F) of computed tomography angiographies from the patient in case 2. (A), (B), and (C) After the first dissection a single dissection flap in the ascending aorta, arch, and descending aorta separate the true (T) and false (F) lumens. (D), (E), and (F) Corresponding images from when the patient presented 5 years later with a recurrent dissection demonstrating the appearance of a second false lumen (F2) and the original false lumen (F1). The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 4 (A) and (C) Planar reformats in the coronal projection and axial images (B) and (D) of computed tomography angiographies from the patient in case 3. (A) and (B) When the patient presented with the initial aortic dissection, a single dissection flap was observed extending from the ascending aorta to the proximal abdominal aorta. (C) and (D) A subsequent scan after the recurrent dissection that demonstrates a triple barrel aortic lumen in the proximal and distal descending thoracic aorta. Note the interposition ascending thoracic graft in (D). The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 5 (A), (B), (D), and (E) Axial images and (C) and (F) planar reformats in the left-anterior oblique projection of computed tomography angiographies from the patient in case 4. (A), (B), and (C) After repair of the ascending thoracic aorta after the first dissection, demonstrating a single dissection flap in the arch and descending aorta separate the true (T) and false (F) lumens. (D), (E), and (F) Corresponding images from when the patient presented with a recurrent dissection demonstrating the appearance of a second false lumen (F2) with shrinking of the original false lumen (F1). The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 6 Sagittal view of the computed tomography angiographies from the patient in case 5. The aortic root (R) is visible adjacent to the artifact associated with the prosthetic valve. Compression of the true lumen (T) and the original false lumen (F1) by the second false lumen (F2) is visible. A dissection flap within F1 is visible in the distal segment. The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 7 Graphical representation of aneurysm measurements before and after the double dissection event. All patients experienced an increase in aortic diameter with an average of 1.6 ± 0.3 cm. The Annals of Thoracic Surgery 2015 99, 1616-1623DOI: (10.1016/j.athoracsur.2014.12.066) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions