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[Clinical and morphological parallels in mitral valve disorders in infants with atrioventricular defect]

T V Sukhaheva, B U Abduvokhidov, A S Lashneva

    Vestnik Rossiiskoi Akademii Meditsinskikh Nauk
    |December 18, 2012
    PubMed

    Insights

    Myxomatous degeneration in pediatric mitral valves, linked to atrioventricular defects, indicates poor surgical outcomes. This tissue change is a key indicator for future mitral valve regurgitation.

    Area of Science:

    • Cardiovascular Pathology
    • Pediatric Cardiology
    • Surgical Pathology

    Background:

    • Atrioventricular defects (AVDs) are congenital heart conditions requiring surgical intervention.
    • Residual mitral valve regurgitation (MVR) can occur post-correction, necessitating further evaluation.
    • Mitral valve tissue characteristics in pediatric AVDs are not fully understood.

    Purpose of the Study:

    • To investigate the histological and ultrastructural features of mitral valve tissue in children with complete and partial atrioventricular defects.
    • To determine the correlation between myxomatous degeneration and the severity of mitral valve regurgitation.
    • To assess the prognostic value of myxomatous degeneration in patients undergoing mitral valve surgery for AVDs.

    Main Methods:

    • Histological examination of mitral valve tissue samples from 19 pediatric patients with AVDs.
    • Ultrastructural analysis to classify cell types within the mitral valve tissue.
    • Immunohistochemistry to determine cell phenotypes, including fibroblastic, endothelial, and smooth muscle differentiation.

    Main Results:

    • Myxomatous degeneration was identified in 32% of patients, occupying over 50% of the mitral valve surface.
    • Areas of myxomatous degeneration showed dispersed star-shaped cells, disturbed architecture, acid mucopolysaccharide deposition, and increased matrix metalloproteinase 13.
    • The extent of myxomatous degeneration correlated with the grade of mitral valve regurgitation and was associated with a higher rate of suture dehiscence post-surgery, indicating a poor prognosis.

    Conclusions:

    • Myxomatous degeneration is a significant pathological finding in pediatric mitral valves associated with atrioventricular defects.
    • Wider areas of myxomatous degeneration predict poorer surgical outcomes, including increased risk of suture dehiscence and residual mitral valve regurgitation.
    • The cellular composition includes fibroblasts, myofibroblasts, and isolated histiocytes, with varying degrees of fibroblastic, endothelial, and smooth muscle differentiation.

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