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J Thorac Cardiovasc Surg 2007;133:1226-1233
© 2007 The American Association for Thoracic Surgery

Surgery for Acquired Cardiovascular Disease

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

Hans-H. Sievers, MD*, Claudia Schmidtke, MD, MBA

Klinik für Herzchirurgie, Universitaetsklinikum Schleswig-Holstein, Campus Luebeck, Luebeck, Germany.

Received for publication August 10, 2006; revisions received January 10, 2007; accepted for publication January 23, 2007.

* Address for reprints: Professor Dr med Hans-H. Sievers, Klinik für Herzchirurgie UKSH, Campus Luebeck, Ratzeburger Allee 160, 23538 Luebeck, Germany. (Email: h.sievers{at}herzchirurgie-luebeck.de).

Objective: In general, classification of a disease has proven to be advantageous for disease management. This may also be valid for the bicuspid aortic valve, because the term "bicuspid aortic valve" stands for a common congenital aortic valve malformation with heterogeneous morphologic phenotypes and function resulting in different treatment strategies. We attempted to establish a classification system based on a 5-year data collection of surgical specimens.

Methods: Between 1999 and 2003 a precise description of valve pathology was obtained from operative reports of 304 patients with a diseased bicuspid aortic valve. Several different characteristics of bicuspid aortic valves were tested to generate a pithy and easily applicable classification system.

Results: Three characteristics for a systematic classification were found appropriate: (1) number of raphes, (2) spatial position of cusps or raphes, and (3) functional status of the valve. The first characteristic was found to be the most significant and therefore termed "type." Three major types were identified: type 0 (no raphe), type 1 (one raphe), and type 2 (two raphes), followed by two supplementary characteristics, spatial position and function. These characteristics served to classify and codify the bicuspid aortic valves into three categories. Most frequently, a bicuspid aortic valve with one raphe was identified (type 1, n = 269). This raphe was positioned between the left (L) and right (R) coronary sinuses in 216 (type 1, L/R) with a hemodynamic predominant stenosis (S) in 119 (type 1, L/R, S). Only 21 patients had a "purely" bicuspid aortic valve with no raphe (type 0).

Conclusions: A classification system for the bicuspid aortic valve with one major category ("type") and two supplementary categories is presented. This classification, even if used in the major category (type) alone, might be advantageous to better define bicuspid aortic valve disease, facilitate scientific communication, and improve treatment.

Abbreviations and Acronyms B = balanced insufficiency and stenosis; BAV = bicuspid aortic valve; I = insufficiency; L = left; No = no insufficiency and stenosis; R = right; S = predominant stenosis; X = nonclassifiable




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