Eisenmenger’s syndrome classification: Difference between revisions

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


[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
* Diller et al. classified patients with [[Eisenmenger's syndrome]] according to the underlying [[congenital heart disease]] into<ref name="pmid17325254">{{cite journal| author=Diller GP, Gatzoulis MA| title=Pulmonary vascular disease in adults with congenital heart disease. | journal=Circulation | year= 2007 | volume= 115 | issue= 8 | pages= 1039-50 | pmid=17325254 | doi=10.1161/CIRCULATIONAHA.105.592386 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17325254  }}</ref>:


*[Group1]
**Simple: [[Atrial septal defect|ASD]], [[Ventricular septal defect|VSD]], persistent [[ductus arteriosus]], and [[aortopulmonary window]].
*[Group2]
**Complex: [[Pulmonary atresia]] with [[Ventricular septal defect|VSD]], [[tricuspid atresia]], [[atrioventricular septal defect]], and [[tetralogy of Fallot]].
*[Group3]
 
*[Group4]
* According to the histopathologic criteria of Heath and Edwards, there are six stages of pulmonary vascular disease (including [[Eisenmenger's syndrome]])<ref name="pmid13573570">HEATH D, EDWARDS JE (1958) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=13573570 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. [http://dx.doi.org/10.1161/01.cir.18.4.533 DOI:10.1161/01.cir.18.4.533] PMID: [https://pubmed.gov/13573570 13573570]</ref>:
 
** Stage I: Medial [[Hypertrophy (medical)|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==
==References==

Latest revision as of 14:54, 26 January 2020

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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

  • 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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