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. 2011;38(6):663-6.

Aggressive aortic replacement for Loeys-Dietz syndrome

G Chad Hughes  1
Affiliations

Aggressive aortic replacement for Loeys-Dietz syndrome

G Chad Hughes. Tex Heart Inst J. 2011.
No abstract available

Keywords: Abnormalities, multiple/genetics/therapy; Loeys-Dietz syndrome/diagnosis/genetics/pathology/surgery; aneurysm, dissecting/genetics/surgery; aortic aneurysm/genetics/surgery; blood vessel prosthesis implantation/methods; cardiovascular system/growth & development.

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Figures

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Fig. 1 Contrast-enhanced computed tomographic angiographic (CTA) images of a Loeys-Dietz patient who underwent valve-sparing aortic root replacement (VSRR) at age 16 for root aneurysm. A) CTA 5 years after VSRR shows mild dilation of the residual native ascending aorta above the Dacron root graft to a diameter of 3.8 cm. B) CTA 8 years after VSRR shows further enlargement to 4.2 cm. C) Aorta measures 4.9 cm by CTA 10 years after VSRR. Surgical repair was recommended at this point but the patient requested delay for several months for personal reasons. D) Coronal and E) axial CTA images 6 months after scan shown in panel C show new acute type A aortic dissection of the residual native ascending aorta with further dilation of the aorta to 6.5 cm, necessitating emergent repair.
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Fig. 2 A) Three-dimensional computed tomographic angiographic (CTA) reconstruction of the aorta at the time of acute type A dissection in a Loeys-Dietz patient and B) axial CTA image 2 months after type A dissection repair show rapid enlargement of the dissected descending aorta in a short interval.
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Fig. 3 A) Anteroposterior and B) lateral view 3-dimensional computed tomographic angiographic reconstructions of the aorta after “hybrid” repair in a patient with Loeys-Dietz syndrome (LDS). Six years after aortic root replacement for repair of an acute type A dissection at age 45, the patient underwent Crawford extent III replacement of a thoracoabdominal aortic aneurysm (TAAA) that enlarged secondary to chronic dissection. A year later, repeat sternotomy for total aortic arch replacement via the elephant-trunk technique was performed for an enlarging transverse arch/proximal descending aneurysm; to complete treatment of the LDS aortic disease, staged endovascular exclusion of the remainder of the chronically dissected aorta was performed during the same hospitalization. Long-segment landing zones for the endografts lay proximally within the Dacron elephant-trunk graft and distally within the Dacron TAAA graft above the takeoff of the side branch to the celiac axis. A hybrid technique was chosen because of the patient's severe scoliosis in union with an extremely tortuous descending aorta that crossed over into the right side of the chest during part of its course.
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