Pedro J. del Nido, Christopher Baird 

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Congenital Mitral Valve Stenosis: Anatomic Variants and Surgical Reconstruction  Pedro J. del Nido, Christopher Baird  Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual  Volume 15, Issue 1, Pages 69-74 (January 2012) DOI: 10.1053/j.pcsu.2012.01.011 Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 1 Mitral valve development from early endocardial cushions to mature valve formation. (from Layman and Edwards4) Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 2 Typical congenital mitral stenosis with marked reduction of the space between the anterolateral (ALP) and the posteromedial (PMP) papillary muscle groups, which are identifiable but fused. Both papillary muscle groups are present and both receive insertions from the shortened and thickened chordae tendineae, but the anomaly may be regarded as a forme fruste of parachute mitral valve. The mitral valve orifice is markedly reduced; there is only one small opening above the interpapillary muscle space (IPMS) and another small aperture at the anterolateral commissure (ALC) (from Ruckman and Van Praagh1). FR, fibrous ring. Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 3 (A) Exposure to the mitral valve is shown via a trans-septal approach. The atrial surface of both mitral leaflets and the thickened ring of tissue restricting the leaflet motion and fusing the commissures can be seen. (B) Supravalve mitral ring. Resection by either sharp or occasionally blunt dissection developing a plane between the leaflet and the ring of connective tissue can be achieved by releasing the leaflets and separating the adherent leaflets at their commissures (top); leaflet commissures are more clearly defined once the adherent layer of fibroelastic tissue is removed (bottom). Ao, aorta; SVC, superior vena cava; RA, right atrium; IVC, inferior vena cava; MV, mitral valve. Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 4 Posterior leaflet retracted by suture to place tension on the tethering connective tissue that needs to be removed. Similar traction can be used on the anterior leaflet to facilitate removal of adherent connective tissue on the edge of the leaflet. Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 5 Congenital MS with a combination of a connective tissue layer on the valve leaflets, short fused chords, multiple papillary muscles, and accessory chords tethering the posterior leaflet. To gain access to the subvalvar structures, detachment of the posterior leaflet close to the annulus facilitates access to the accessory chords and papillary muscles. The incision needs to extend from commissure to commissure. Aggressive delamination of the papillary muscles leaving only the apical-most attachments helps mobilize these structures for improved leaflet mobility. Inset, lower right: cross-sectional view of the leaflet incision and papillary muscle dissection. LA, left atrium; LV, left ventricle. Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 6 Extension of the posterior leaflet can be achieved with autologous treated pericardium or similar xenograft material such as porcine intestinal submucosa or “matrix” patch. Care must be taken not to extend the patch completely to the commissures because this can result in folding of the patch limiting its usefulness. Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 7 Freedom from reoperation in children following mitral valve reconstructive surgery shown by primary indication for surgery. Mixed lesion with significant mitral valve stenosis and regurgitation (left). Freedom from reoperation shown by age group (right). MR, mitral valve regurgitation; MS, mitral valve stenosis; MS/MR, mixed lesion with significant mitral valve stenosis and regurgitation. Seminars in Thoracic & Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2012 15, 69-74DOI: (10.1053/j.pcsu.2012.01.011) Copyright © 2012 Elsevier Inc. Terms and Conditions