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April 13, 2021
Degenerative Disease Alain Carpentier

Alain Carpentier (1933- )

Carpentier A, et al . Reconstructive surgery of mitral valve incompetence: Ten-year appraisal. J Thorac Cardiovasc Surg 1980;79(3):338-48

Alain Carpentier
Portrait of Alain Carpentier
Portrait of Alain Carpentier
Cardiac valve surgery- the "French correction". J Thorac Cardiovasc Surg 1983; 86:323-337

Cesalpino Andrea
Cesalpino Andrea

After Reid's and Barlow's initial work, several reports were published to describe this new clinical entity, "mitral regurgitation with mid systolic click and late apical systolic murmur." Various terminologies were used to describe this condition based on auscultatory findings (chordal snap, click syndrome) or on pathological findings (myxomatous valve, myxoid valve, floppy valve) or on cineangiographical findings (Ballooning valve, billowing posterior leaflet, prolapse leaflet).

Criley in his article of 1966 wrote "the difference between billowing and prolapse may merely be semantic or only a matter of degree, with one condition merging into another." In contrast, Barlow was of the opinion that "This [use of multiple terminologies] has contributed to the general confusion and it seems to us that it is more than a matter of semantics for terms such as billowing, prolapse, floppy, or flail to be better defined." Barlow then continued "The concept propounded by the French cardiac surgeon, Alain Carpentier, defines the essential and important differences between mitral valve prolapse and billowing mitral leaflets."

Barlow was obviously referring to Carpentier's Pathophysiological triad and functional classification.

Carpentier introduced the concept of pathophysiological triad to make a clear distinction between terminologies describing the etiology (the cause of the valve disease), lesions (resulting from the disease) and dysfunction (resulting from the lesions).

 The functional approach, first described in 1978, was based on the analysis of the motion of the leaflets during diastole and systole. Three functional types were distinguished depending upon whether the leaflet motion is normal (type I) increased (leaflet prolapse or type II) or decreased (restricted leaflet motion or type III).

Cesalpino Andrea

Using this simplified approach, the etiology of this degenerative valve disease with excess leaflet tissue, billowing valves, and myxomatous degeneration on histology was named Barlow's disease because of the seminal contributions of this author. Leaflet billowing was described as a valvular lesion and leaflet prolapse as a dysfunction indicating excess motion of one or both leaflets of the mitral valve overriding the plane of the annulus during systole.

Carpentier's extensive clinical and operative experience led him to define a second type of degenerative mitral valve disease, Fibroelastic deficiency, in 1975. Contrary to Barlow's disease, Carpentier remarked that this degenerative disease was characterized by extremely thin leaflets and chordae without excess leaflet tissue. Furthermore, fibroelastic deficiency had a limited time course, no familial forms and was often diagnosed in elderly patients. The mechanism of mitral regurgitation in most patients was an isolated prolapse of the middle scallop of the posterior leaflet due to chordae rupture.

In 1981, Carpentier and his colleagues published a landmark article comparing the clinical, pathological, and histological characteristics of Barlow's disease and fibroelastic deficiency:

A) Barlow's disease :

  • Average age at operation-48 years. Average time interval between first clinical symptom and operation -28 years

  • Redundant, thickened, and yellowish leaflets with excess tissue.  Severe dilatation of the annulus. Elongated and irregular (thin or thickened) chordae.

  • Disruption of collagen and elastic layers, and excess acid mucopolysaccharides.

B) Fibroelastic deficiency :

  • Average age at operation-61 years. Average time interval between first clinical symptom and operation-6 years

  • Smooth, thin, and translucent leaflets (no excess tissue). Moderate dilatation of annulus. Slightly elongated and very thin chordae.

  • Fibroelastic and acid mucopolysaccharide deficiency.

Cesalpino Andrea

Cesalpino Andrea


Michael Criley