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Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature.

Publication ,  Journal Article
Rajagopal, K; Rogers, JG; Lodge, AJ; Gaca, JG; McCann, RL; Milano, CA; Hughes, GC
Published in: J Heart Lung Transplant
September 2009

A 24-year-old man with Marfan syndrome underwent mitral valve repair for prolapse at age 13. He sustained an acute type A aortic dissection at age 20 and underwent aortic root/ascending aortic replacement with a mechanical valved conduit. He initially did well after the latter procedure, but end-stage heart disease developed 4 years later, apparently secondary to primary cardiomyopathy. Pre-transplant evaluation revealed residual chronic dissection with aneurysmal dilatation of the distal ascending aorta, transverse arch, and descending thoracic aorta. He underwent combined orthotopic heart transplantation (OHT) and total arch replacement (stage I elephant trunk procedure). Subsequently, he underwent extent II thoracoabdominal aneurysm repair, leaving no residual aortic disease. The 2 procedures resulted in total cardioaortic replacement, thus definitively managing his cardiomyopathy and aortic disease resulting from Marfan syndrome. The operative strategies employed represent a novel approach in this clinical setting. This report emphasizes that patients with this disease should not be denied potentially life-saving OHT on the basis of concomitant aortic disease, but rather should be treated in centers offering expertise in both areas of surgical therapy.

Duke Scholars

Published In

J Heart Lung Transplant

DOI

EISSN

1557-3117

Publication Date

September 2009

Volume

28

Issue

9

Start / End Page

958 / 963

Location

United States

Related Subject Headings

  • Young Adult
  • Treatment Outcome
  • Surgery
  • Mitral Valve
  • Marfan Syndrome
  • Male
  • Humans
  • Heart Valve Prosthesis Implantation
  • Heart Transplantation
  • Heart Failure
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Rajagopal, K., Rogers, J. G., Lodge, A. J., Gaca, J. G., McCann, R. L., Milano, C. A., & Hughes, G. C. (2009). Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature. J Heart Lung Transplant, 28(9), 958–963. https://doi.org/10.1016/j.healun.2009.05.012
Rajagopal, Keshava, Joseph G. Rogers, Andrew J. Lodge, Jeffrey G. Gaca, Richard L. McCann, Carmelo A. Milano, and G Chad Hughes. “Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature.J Heart Lung Transplant 28, no. 9 (September 2009): 958–63. https://doi.org/10.1016/j.healun.2009.05.012.
Rajagopal K, Rogers JG, Lodge AJ, Gaca JG, McCann RL, Milano CA, et al. Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature. J Heart Lung Transplant. 2009 Sep;28(9):958–63.
Rajagopal, Keshava, et al. “Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature.J Heart Lung Transplant, vol. 28, no. 9, Sept. 2009, pp. 958–63. Pubmed, doi:10.1016/j.healun.2009.05.012.
Rajagopal K, Rogers JG, Lodge AJ, Gaca JG, McCann RL, Milano CA, Hughes GC. Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature. J Heart Lung Transplant. 2009 Sep;28(9):958–963.
Journal cover image

Published In

J Heart Lung Transplant

DOI

EISSN

1557-3117

Publication Date

September 2009

Volume

28

Issue

9

Start / End Page

958 / 963

Location

United States

Related Subject Headings

  • Young Adult
  • Treatment Outcome
  • Surgery
  • Mitral Valve
  • Marfan Syndrome
  • Male
  • Humans
  • Heart Valve Prosthesis Implantation
  • Heart Transplantation
  • Heart Failure