Aorta Complications After AVR in Bicuspid Aortic Valve vs. Marfan | Journal Scan

Study Questions:

Is the natural history of thoracic aortopathy after aortic valve replacement (AVR) in patients with bicuspid aortic valve disease substantially different from that seen in patients with Marfan syndrome?


In a retrospective comparison, outcomes were compared for 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. The median follow-up time was 6.6 years.


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 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%, p< 0.001) compared with those with bicuspid valves (4.8 ± 0.8%) and control patients (1.4 ± 0.2%). Patients with Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%, p < 0.001) compared with those with bicuspid valves (2.5 ± 0.6%) and control patients (0.50 ± 0.09%).


The much higher long-term rates of aortic complications after AVR observed in patients with Marfan syndrome compared with those with bicuspid aortic valve 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.


In addition to valve dysfunction, bicuspid aortic valves are associated with ascending aortic aneurysm and aortic dissection (bicuspid aortopathy). Management of the ascending aorta at the time of AVR in patients with bicuspid aortic valve is controversial. This important study suggests that bicuspid aortopathy has fewer aortic complications compared to patients with Marfan syndrome, and that guidelines for management should be specific to the underlying condition rather than extrapolated from the Marfan population.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Structural Heart Disease, Interventions and Vascular Medicine

Keywords: Algorithms, Aortic Aneurysm, Thoracic, Aortic Aneurysm, Aortic Valve, Bicuspid, Cardiac Surgical Procedures, Heart Defects, Congenital, Heart Valve Diseases, Incidence, Marfan Syndrome, Mitral Valve, Retrospective Studies

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