New morphological classification of congenital quadricuspid aortic valve and its histopathologic features

Cardiovasc Pathol. 2018 Jul-Aug:35:8-11. doi: 10.1016/j.carpath.2018.03.005. Epub 2018 Apr 6.

Abstract

We report a 52-year-old male patient who had a quadricuspid aortic valve (QAV) associated with aortic regurgitation (AR) and left ventricular hypertrophy (LVH). A new accessory cusp (ACC) with maximum thickness than other cusps was located between right coronary cusp (RCC) and left coronary cusp (LCC). The histopathological features revealed markedly thickened and distorted cusp architecture with fibrosis and/or myxomatous degeneration in both non-coronary cusp (NCC) and ACC. Two equal sizes for larger cusps (RCC and NCC) and two equal sizes for smaller cusps (LCC and ACC) were obtained. This QAV belonged to type C QAV of Hurwitz's classification, but also suggested as a modified type III of Jagannath's classification or a new type V of Nakamura's classification by locating ACC between RCC and LCC.

Keywords: Aortic regurgitation (AR); Morphological classification; Quadricuspid aortic valve (QAV).

Publication types

  • Case Reports

MeSH terms

  • Aortic Valve / abnormalities*
  • Aortic Valve / physiopathology
  • Aortic Valve / transplantation
  • Aortic Valve Insufficiency / pathology*
  • Aortic Valve Insufficiency / physiopathology
  • Aortic Valve Insufficiency / surgery
  • Biopsy
  • Echocardiography, Doppler, Color
  • Echocardiography, Transesophageal
  • Fibrosis
  • Heart Defects, Congenital / classification
  • Heart Defects, Congenital / pathology*
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery
  • Heart Valve Prosthesis Implantation
  • Humans
  • Hypertrophy, Left Ventricular / pathology*
  • Hypertrophy, Left Ventricular / physiopathology
  • Hypertrophy, Left Ventricular / surgery
  • Male
  • Middle Aged
  • Treatment Outcome