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Postoperative Thoracic Aorta in the Marfan Patient: Normal Postoperative Appearance and Spectrum of Complications

Introduction

Historically conventional cardiovascular surgical treatment for patients with the Marfan syndrome who have aneurysm or dissection involving the ascending aorta has been replacement of the entire aortic root
Current therapy for patients with a dilated aortic root and Marfan syndrome is either replacement of the entire aortic root and valve with a composite graft and mechanical or biological valve or valve-sparing operation
The native coronary arteries are reimplanted into the graft
In this exhibit we will review both the normal postoperative appearance as well as the spectrum of postoperative complications ranging from dissection to pseudoaneurysm formation to coronary artery aneurysms

Marfan syndrome

Connective tissue disorder
Mutation of the fibrillin-1 gene
Autosomal dominant
25-30% sporadic mutations
Wide range of clinical manifestations including cardiovascular, ocular, musculoskeletal, central nervous system, and pulmonary

Marfan Syndrome

Ocular
- Ectopia lentis, retinal detachment
Musculoskeletal
- Scoliosis, pectus excavatum and carinatum, arachnodactyly, acetabular protrusion
Central nervous system
- Dural ectasia
Pulmonary
- Spontaneous pneumothorax, bullae

Marfan Syndrome

Cardiovascular manifestations

Annuloaortic ectasia +/- aortic valvular insufficiency
Aortic dissection
Aortic aneurysm
Pulmonary artery dilatation
Mitral valve prolapse

Dilated Aortic Root

68 year old female with no history of surgical repair. 3D maps and reformatted images show dilatation of the supravalvular aortic root and ascending aorta characteristic of annuloaortic ectasia. Operative repair is indicated when aorta reaches a diameter of 5 cm due to high risk of rupture. Classic “tulip bulb” configuration.

Cardiovascular manifestations

Aortic dissection, congestive heart failure, and cardiac valve disease are the most common causes of death in >90% of those affected
However, in the past 30 years, improvements in diagnostic techniques and in therapy (both medical and surgical) have led to a considerable increase in life expectancy
Common surgical techniques include Bentall procedure, modified Bentall procedure, and David I inclusion procedure

Post-operative complications

By definition residual or recurrent disease after initial repair
Overall there is a low complication rate with these surgical techniques
Complications may include the following:
- Expanding aneurysm in a previously dissected but not resected aortic segment
- Pseudoaneurysms or leakage at the suture lines of the aortic or coronary anastomosis
- Ongoing degeneration of the aortic valve leaflets following a valve-sparing root repair
- Aneurysm at the site of coronary artery reimplantation
- Abnormal accumulation of low-attenuation material around the graft or collection of contrast material outside the graft

Case Studies

The following cases represent patients with Marfan syndrome following surgical repair of the thoracic aorta
The initial cases show normal postoperative appearance with both composite graft and prosthetic valve as well as valve-sparing procedure
Subsequently we demonstrate various postoperative complications encountered at our institution

Case 1:Normal postoperative appearance in patient with composite graft and mechanical aortic valve replacement

19 year old female presented for routine follow up evaluation. She has had prior aortic root replacement with composite graft as well as aortic and mitral valve replacement. Images A and B are sagittal reformations demonstrating artifact related to prosthetic aortic and mitral valves. Image C best shows the “waisting” of the ascending aorta due to composite graft placement.

Case 2:Normal postoperative appearance in patient with valve-sparing operation

40 year old male with prior valve-sparing aortic root replacement. No complications seen. Note absence of valve replacement. The 3D maps (images A and B) best demonstrate the contour defect related to surgical repair and area of coronary artery re-implantation into the graft. Image C shows suture lines related to vascular graft.

Case 3:Normal postoperative appearance in patient with valve-sparing operation

36 year old female with prior valve-sparing aortic root replacement. No complications seen. Axial and sagittal reformatted images demonstrate placement of vascular graft within the native aortic root and ascending aorta. Note absence of aortic valve replacement.

Case 3:Normal postoperative appearance in patient with valve-sparing operation

Continued… 3D color maps show contour defect related to surgical repair and vascular graft placement. See arrows. The right coronary artery re-implantation site is also well visualized.

Case 4:Dissection following aortic root replacement

40 year old male presented with chest pain. Patient had history of aortic root replacement with composite graft. Reformatted axial and sagittal CT images demonstrated focal Type A dissection of the aortic arch - limited to the aortic arch - above the level of the composite graft which terminated at the origin of the left subclavian artery. Note artifact related to prosthetic aortic valve.

Case 4:Dissection following aortic root replacement

Continued… Sagittal reformatted images and sagittal 3D color map show dilatation of the origin of the coronary arteries at the site of implantation. See arrows. Again seen is artifact related to aortic and mitral valve replacement.

Case 5:Dissection following aortic root replacement

39 year old male presented for routine follow up imaging. History of aortic root replacement with composite vascular graft. CT reformatted images as well as 3D maps show Type A dissection beginning just above the distal end of the composite graft continuing through the aortic arch and descending thoracic aorta. Note artifact related to prosthetic aortic valve. Contour defect related to graft placement best seen on the color maps.

Case 6:Coronary artery aneurysms at site of implantation

19 year old female presented for routine follow up imaging. History of aortic root replacement with composite graft. See images of normal postoperative appearance in Case 1. Axial reformatted images demonstrate dilatation of the origin of the right coronary artery just at the level of the anastomosis shown best on 3D mapping. See next slide. Also mild dilatation of the origin of the left main coronary artery. Vessel are of normal caliber distally.

Case 6:Coronary artery aneurysms at site of implantation

Case 7:Coronary artery aneurysms at site of implantation

38 year old female with history of valve-sparing aortic root replacement in 2005. On routine follow up echocardiogram found to have right coronary artery aneurysm. Reformatted images in axial and sagittal planes show focal dilatation of the origin of the right coronary artery at the anastomotic site.

Case 7:Coronary artery aneurysms at site of implantation

Continued… 3D color maps show focal asymmetric dilatation of the origin of the right coronary artery at the anastomotic site. Abnormality seen in all three planes. See arrows.

Case 8:Mediastinal fluid collection 2 years after surgery

34 year old male admitted for chest pain and fever. 2 years prior to admission had aortic root replacement with composite graft. Note artifact related to aortic valve replacement. CT showed perigraft low attenuation material measuring 45-55 HU which exerts mild mass effect on right main pulmonary artery. No evidence of contrast leak or active extravasation. No pseudoaneurysm. 3D map better delineates the vascular graft and coronary artery implantation site.

Case 9:Dilatation of the aortic sinuses and aortic root

38 year old male presented with chest pain. Evidence of prior aortic root replacement with composite graft. See arrows delineating the vascular graft itself. This is best seen on the 3D color maps.

Case 9:Dilatation of the aortic sinuses and aortic root

Continued… Reformatted images show dilatation of the aortic root below the level of the composite graft.

Conclusion

In summary, the results of postoperative imaging evaluation after ascending aortic surgery have been analyzed and impact surgical decision making
Precise knowledge of the surgical technique performed and its anatomic consequences is crucial to the accurate postoperative imaging evaluation
This knowledge allows one to discern normal postoperative findings from those indicative of a postoperative complication, possibly requiring intervention
Clinical evaluation is limited because it does not enable detection of the early stages of subtle complications, before development of the acute signs and symptoms that indicate an emergent situation
Therefore postoperative assessment by means of imaging including CT (also MRI and angiography) has been pursued

References

Kallenbach K, Karck M, Haverich A. Valve-sparing aortic root replacement: the inclusion (David) technique. Multimedial Manual of Cardiothoracic Surgery 2007; 2007 (0507): 1917.
Miller DC. Valve-sparing aortic root replacement in patients with the Marfan syndrome. J Thorac Cardiovasc Surg 2003;125: 773–778.
Karck M, Kallenbach K, Hagl C, Rhein C, Leyh R, Haverich A. Aortic root surgery in Marfan syndrome: comparison of aortic valve sparing reimplantation versus composite grafting. J Thorac Cardiovasc Surg 2004;127: 391–398.
Gott VL, Greene PS, Alejo DE, Cameron DE, Naftel DC, Miller DC, et al. Replacement of the aortic root in patients with Marfan's syndrome. N Engl J Med. 1999;340: 1307-13.
Carrel T, et al. Reoperations and late adverse outcome in Marfan patients following cardiovascular surgery. European Journal of Cardio-thoracic surgery 2004; 25: 671-675.

References

Rofsky NM, Weinreb JC, Grossi EA, et al. Aortic Aneurysm and Dissection: Normal MR Imaging and CT Findings after Surgical Repair with the Continuous-Suture Graft-Inclusion Technique. Radiology 1993; 186: 195-201.
Sundaram B, Quint LE, Patel S, Patel HJ, Deeb GM. CT Appearance of Thoracic Aortic Graft Complications. American Journal of Roentgenology 2007; 188: 1273-1277.

Acknowledgements:
A A Chudgar MD
D Cameron MD
P T Johnson MD
K M Horton MD
F M Corl MS
E K Fishman MD