A classification system for the bicuspid aortic valve from 304 surgical specimens

Area of Science:

• Cardiovascular Medicine
• Congenital Heart Disease
• Surgical Pathology

Background:

• Bicuspid aortic valve (BAV) is a common congenital malformation with diverse presentations.
• Heterogeneous phenotypes and functions of BAV necessitate refined classification for effective management.
• Current classification challenges hinder precise disease definition and treatment strategies.

Purpose of the Study:

• To establish a novel, practical classification system for bicuspid aortic valve disease.
• To categorize BAV based on objective morphological and functional characteristics.
• To facilitate improved scientific communication and clinical decision-making for BAV.

Main Methods:

• Analysis of surgical specimens from 304 patients with diseased bicuspid aortic valves (1999-2003).
• Evaluation of various BAV characteristics, including raphe number, cusp/raphe spatial position, and valve function.
• Development of a classification system based on significant pathological findings.

Main Results:

• A classification system based on three key characteristics was identified: number of raphes (type), spatial position, and function.
• Three major types were defined: type 0 (no raphe), type 1 (one raphe), and type 2 (two raphes).
• Type 1 (one raphe) was most frequent (n=269), often positioned between left/right coronary sinuses (n=216) and associated with stenosis (n=119).

Conclusions:

• A new classification system for bicuspid aortic valve disease, featuring a primary 'type' category and supplementary characteristics, has been proposed.
• This classification system can enhance the definition of BAV disease.
• Implementation of this system may improve scientific discourse and optimize patient treatment strategies.