The bicuspid aortic valve (BAV), the most common congenital heart defect, is characterized by two aortic valve cusps rather than the normal three. BAV anatomy exhibits significant variability, with classifications based on cusp fusion patterns (e.g., right-left [RL], right-non-coronary [R-NC], left-non-coronary [L-NC]), presence of a raphe, and cusp orientation. Multiple classification systems have been proposed, including the Schäfers, Sievers, and BAVCon systems, each highlighting different aspects of BAV morphology. Advanced imaging methods like echocardiography and computed tomography are integral part in diagnosing and categorizing BAV phenotypes, influencing surgical and interventional planning. BAV is also associated with unique coronary artery patterns, such as higher rates of left-dominant systems and variable coronary ostia positioning, which carry implications for transcatheter and surgical procedures. Additionally, BAV is linked to conduction system abnormalities, increasing the risk of permanent pacemaker (PPM) implantation following interventions. Understanding the anatomical and morphological variations of BAV is critical for predicting complications such as stenosis, regurgitation, and aortic aneurysms, tailoring management strategies, and optimizing procedural outcomes.

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Morphology Spectrum of Bicuspid Aortic Valve and Atrio-Ventricular Conduction System

  • Chiara Fraccaro,
  • Cristina Basso,
  • Gaetano Thiene

摘要

The bicuspid aortic valve (BAV), the most common congenital heart defect, is characterized by two aortic valve cusps rather than the normal three. BAV anatomy exhibits significant variability, with classifications based on cusp fusion patterns (e.g., right-left [RL], right-non-coronary [R-NC], left-non-coronary [L-NC]), presence of a raphe, and cusp orientation. Multiple classification systems have been proposed, including the Schäfers, Sievers, and BAVCon systems, each highlighting different aspects of BAV morphology. Advanced imaging methods like echocardiography and computed tomography are integral part in diagnosing and categorizing BAV phenotypes, influencing surgical and interventional planning. BAV is also associated with unique coronary artery patterns, such as higher rates of left-dominant systems and variable coronary ostia positioning, which carry implications for transcatheter and surgical procedures. Additionally, BAV is linked to conduction system abnormalities, increasing the risk of permanent pacemaker (PPM) implantation following interventions. Understanding the anatomical and morphological variations of BAV is critical for predicting complications such as stenosis, regurgitation, and aortic aneurysms, tailoring management strategies, and optimizing procedural outcomes.