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

Eisenmenger's syndrome can be classified according to the underlying congenital heart disease into simple and complex. Histologically, six stages were identified by Heath and Edwards, ranging from reversible to irreversible and terminal.

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)