Eisenmenger’s syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

Overview

[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

Classification

• Diller et al. classified patients with Eisenmenger's syndrome according to the underlying congenital heart disease into:

• • Simple: ASD, VSD, persistent ductus arteriosus, and aortopulmonary window.
  • Complex: Pulmonary atresia with VSD, tricuspid atresia, atrioventricular septal defect, and tetralogy of Fallot.

• According to the histopathologic criteria of Heath and Edwards, there are six stages of pulmonary vascular disease (including Eisenmenger's syndrome):

• • Stage I: Medial hypertrophy (reversible)
  • Stage II: Cellular intimal hyperplasia in an abnormally muscular artery (reversible)
  • Stage III: Lumen occlusion from intimal hyperplasia of fibroelastic tissue (partially reversible)
  • Stage IV: Arteriolar dilatation and medial thinning (irreversible)
  • Stage V: Plexiform lesion, which is an angiomatoid formation (terminal and irreversible)
  • Stage VI: Fibrinoid/necrotizing arteritis (terminal and irreversible)