Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome

Shinobu Itagaki; Joanna P Chikwe; Yuting P Chiang; Natalia N Egorova; David H Adams

doi:10.1016/j.jacc.2015.03.575

Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome

J Am Coll Cardiol. 2015 Jun 9;65(22):2363-9. doi: 10.1016/j.jacc.2015.03.575.

Authors

Shinobu Itagaki¹, Joanna P Chikwe², Yuting P Chiang¹, Natalia N Egorova³, David H Adams¹

Affiliations

¹ Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York.
² Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York. Electronic address: [email protected].
³ Department of Health Evidence and Policy, Icahn School of Medicine at Mount Sinai, New York, New York.

PMID: 26046728
DOI: 10.1016/j.jacc.2015.03.575

Abstract

Background: Bicuspid aortic valves are associated with valve dysfunction, ascending aortic aneurysm and dissection. Management of the ascending aorta at the time of aortic valve replacement (AVR) in these patients is controversial and has been extrapolated from experience with Marfan syndrome, despite the absence of comparative long-term outcome data.

Objectives: This study sought to assess whether the natural history of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease is substantially different from that seen in patients with Marfan syndrome.

Methods: In this retrospective comparison, outcomes of 13,205 adults (2,079 with bicuspid aortic valves, 73 with Marfan syndrome, and 11,053 control patients with acquired aortic valve disease) who underwent primary AVR without replacement of the ascending aorta in New York State between 1995 and 2010 were compared. The median follow-up time was 6.6 years.

Results: The long-term incidence of thoracic aortic dissection was significantly higher in patients with Marfan syndrome (5.5 ± 2.7%) compared with those with bicuspid valves (0.55 ± 0.21%) and control group patients (0.41 ± 0.08%, p < 0.001). Thoracic aortic aneurysms were significantly more likely to be diagnosed in late follow-up in patients with Marfan syndrome (10.8 ± 4.4%) compared with those with bicuspid valves (4.8 ± 0.8%) and control group patients (1.4 ± 0.2%) (p < 0.001). Patients with Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%) compared with those with bicuspid valves (2.5 ± 0.6%) and control group patients (0.50 ± 0.09%) (p < 0.001).

Conclusions: The much higher long-term rates of aortic complications after AVR observed in patients with Marfan syndrome compared with those with bicuspid aortic valves confirm that operative management of patients with bicuspid aortic valves should not be extrapolated from Marfan syndrome and support discrete treatment algorithms for these different clinical entities.

Keywords: Marfan syndrome; aortic dissection; ascending aorta; bicuspid aortic valve.

Publication types

Comparative Study

MeSH terms

Adult
Aged
Aortic Aneurysm, Thoracic / epidemiology*
Aortic Aneurysm, Thoracic / etiology
Aortic Dissection / epidemiology*
Aortic Dissection / etiology
Aortic Valve / abnormalities*
Aortic Valve / surgery
Bicuspid Aortic Valve Disease
Female
Follow-Up Studies
Heart Valve Diseases / complications
Heart Valve Diseases / surgery*
Heart Valve Prosthesis Implantation / adverse effects*
Humans
Incidence
Male
Marfan Syndrome / complications*
Middle Aged
New York / epidemiology
Postoperative Complications / epidemiology*
Postoperative Complications / etiology
Retrospective Studies
Risk Factors
Time Factors