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

According to the histopathologic criteria of Heath and Edwards, there are six stages of pulmonary vascular disease (including Eisenmenger's syndrome)[2]:

    • 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)

References

  1. Diller GP, Gatzoulis MA (2007). "Pulmonary vascular disease in adults with congenital heart disease". Circulation. 115 (8): 1039–50. doi:10.1161/CIRCULATIONAHA.105.592386. PMID 17325254.
  2. HEATH D, EDWARDS JE (1958) The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 18 (4 Part 1):533-47. DOI:10.1161/01.cir.18.4.533 PMID: 13573570

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