Bicuspid aortic valve outcomes

Cardiol Young. 2017 Apr;27(3):518-529. doi: 10.1017/S1047951116002560. Epub 2016 Dec 12.


Background: Bicuspid aortic valve is the most common CHD. Its association with early valvular dysfunction, endocarditis, thoracic aorta dilatation, and aortic dissection is well established.

Objective: The aim of this study was to assess the incidence and predictors of cardiac events in adults with bicuspid aortic valve.

Methods: We carried out a retrospective analysis of cardiac outcomes in ambulatory adults with bicuspid aortic valve followed-up in a tertiary hospital centre. Outcomes were defined as follows: interventional - intervention on the aortic valve or thoracic aorta; medical - death, aortic dissection, aortic valve endocarditis, congestive heart failure, arrhythmias, or ischaemic heart disease requiring hospital admission; and a composite end point of both. Kaplan-Meier curves were generated to determine event rates, and predictors of cardiac events were determined by multivariate analysis.

Results: A total of 227 patients were followed-up over 13±9 years; 29% of patients developed severe aortic valve dysfunction and 12.3% reached ascending thoracic aorta dimensions above 45 mm. At least one cardiac outcome occurred in 38.8% of patients, with an incidence rate at 20 years of follow-up of 47±4%; 33% of patients were submitted to an aortic valve or thoracic aorta intervention. Survival 20 years after diagnosis was 94±2%. Independent predictors of the composite end point were baseline moderate-severe aortic valve dysfunction (hazard ratio, 3.19; 95% confidence interval, 1.35-7.54; p<0.01) and aortic valve leaflets calcification (hazard ratio, 4.72; 95% confidence interval, 1.91-11.64; p<0.005).

Conclusions: In this study of bicuspid aortic valve, the long-term survival was excellent but with occurrence of frequent cardiovascular events. Baseline aortic valve calcification and dysfunction were the only independent predictors of events.

Keywords: bicuspid; congenital; Aorta; aortic valve; heart defects; survival.

MeSH terms

  • Adult
  • Aortic Aneurysm, Thoracic / diagnosis
  • Aortic Aneurysm, Thoracic / epidemiology*
  • Aortic Aneurysm, Thoracic / etiology
  • Aortic Valve / abnormalities*
  • Aortic Valve Insufficiency / diagnosis
  • Aortic Valve Insufficiency / epidemiology*
  • Aortic Valve Insufficiency / etiology
  • Aortic Valve Stenosis / diagnosis
  • Aortic Valve Stenosis / epidemiology*
  • Aortic Valve Stenosis / etiology
  • Bicuspid Aortic Valve Disease
  • Cause of Death / trends
  • Disease Progression
  • Echocardiography, Doppler
  • Female
  • Follow-Up Studies
  • Forecasting*
  • Heart Valve Diseases / complications*
  • Heart Valve Diseases / diagnosis
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Outpatients
  • Prognosis
  • Retrospective Studies
  • Survival Rate / trends
  • United States / epidemiology
  • Young Adult