Etiologies of mitral valve prolapse and flail mitral leaflet

Disease	Mechanism of regurgitation
Primary MVP
Nonclassic	Isolated prolapse of the mitral leaflet (commonly P2 scallop) Frequent chordal rupture Mild annular enlargement
Forme fruste Barlow disease	Intermediate features
Classic (Barlow disease)	Diffusely thickened, redundant mitral leaflets Chordal elongation/rupture Severe annular enlargement
Secondary MVP
Associated with connective tissue disease*	Diffusely thickened, redundant mitral leaflets Chordal elongation/rupture Severe annular enlargement
Associated with congenital heart disease^¶	Thickened, redundant mitral leaflets Chordal elongation/rupture possible
Non-MVP flail mitral leaflet
Acute myocardial ischemia	Papillary muscle dysfunction with secondary prolapse/papillary muscle rupture
Acute rheumatic fever	Chordal and leaflet destruction by acute inflammatory process
Endocarditis	Chordal and leaflet destruction by infectious process; vegetations
Other (trauma, severe mitral annular calcification, hypertrophic cardiomyopathy)	Ruptured chordae, no myxomatous changes of mitral valve leaflets

* Associated with Marfan and Ehlers-Danlos syndromes, osteogenesis imperfecta, and pseudoxanthoma elasticum. Macroscopical and microscopical appearance similar to primary Barlow disease.

¶ Most common congenital disease associations are Ebstein's anomaly and atrial septal defect.

Graphic 83655 Version 3.0

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Etiologies of mitral valve prolapse and flail mitral leaflet