Morphologic spectrum of ebstein's malformation: Revisitation relative to surgical repair Christian Schreiber, MD, Andrew Cook, BSc, Siew Yen Ho, PhD,

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Presentation transcript:

Morphologic spectrum of ebstein's malformation: Revisitation relative to surgical repair Christian Schreiber, MD, Andrew Cook, BSc, Siew Yen Ho, PhD, FRCPath, Norbert Augustin, MD, Robert H. Anderson, MD, FRCPath The Journal of Thoracic and Cardiovascular Surgery Volume 117, Issue 1, Pages 148-155 (January 1999) DOI: 10.1016/S0022-5223(99)70480-0 Copyright © 1999 Mosby, Inc. Terms and Conditions

Fig. 1A, The septal and mural leaflets in this heart have not developed. Only the anterosuperior leaflet (AS) has delaminated, but its distal edge is attached to a muscle shelf at the junction between the inlet and apical trabecular portions of the right ventricle. The valvular orifice is at the site of the anteroseptal commissure (arrow). AV, Atrioventricular. B, This view of the right ventricular outlet shows the location of the muscle shelf. The orifice of the tricuspid valve is restricted to the area between the free margin of the anterosuperior leaflet and the ventriculo-infundibular fold. The Journal of Thoracic and Cardiovascular Surgery 1999 117, 148-155DOI: (10.1016/S0022-5223(99)70480-0) Copyright © 1999 Mosby, Inc. Terms and Conditions

Fig. 2A, The septal leaflet in this heart is represented by a cauliflower-like excrescence (star). The anterosuperior leaflet (AS) is sail-like and hinged at the atrioventricular (AV) junction. Tendinous chords at the anteroseptal commissure (arrowhead) attach directly to the ventricular septum. The mural leaflet (M) is adherent to the ventricular wall (arrows) and has a minimal flee edge (V). B, The septal (star) and mural (M) leaflets are formed, but the hinge line is displaced well into the right ventriicular cavity. Note the thin, atrialized ventricular wall (arrows along cut edge). The Journal of Thoracic and Cardiovascular Surgery 1999 117, 148-155DOI: (10.1016/S0022-5223(99)70480-0) Copyright © 1999 Mosby, Inc. Terms and Conditions

Fig. 3A, The mural leaflet (arrowhead) is represented by a fibromuscular flap. The anterosuperior leaflet (AS) has focal attachments, but the valvular orifice is divided by 2 strands of accessory valvular bridges. B, This dissection through the mural leaflet (M) shows its hinge at the atrioventricular (AV) junction. The hinge (dotted line) of the septal leaflet (S) is displaced apically. The Journal of Thoracic and Cardiovascular Surgery 1999 117, 148-155DOI: (10.1016/S0022-5223(99)70480-0) Copyright © 1999 Mosby, Inc. Terms and Conditions

Fig. 4A, The anteroseptal commissure (arrowhead) is not well developed in this heart, but the anterosuperior leaflet is shallow and has focal attachments to the anterior-papillary muscle. B, The anterosuperior leaflet (AS) in this heart is scalloped, with hyphenated attachments distally to a muscle shelf. Multiple interchordal spaces (arrowheads) are present. C, In this heart, the distal edge of the anterosuperior leaflet (AS) is attached linearly to the muscle shelf although a few small perforations are present in the body of the leaflet. The septal leaflet (S) is barely formed. The Journal of Thoracic and Cardiovascular Surgery 1999 117, 148-155DOI: (10.1016/S0022-5223(99)70480-0) Copyright © 1999 Mosby, Inc. Terms and Conditions

Fig. 5A, This view of the right ventricular outflow tract shows the keyhole (arrow) at the site of the anteroseptal commissure. There is linear attachment of the anterosuperior leaflet (AS) to the muscle shelf. B, This heart shows the keyhole (arrow) from the inlet aspect. There is no separation of the distal edges of the mural (M) and anterosuperior (AS) leaflets (between arrowheads). Consequently, the valvular orifice is restricted to the site of the anteroseptal commissure. The Journal of Thoracic and Cardiovascular Surgery 1999 117, 148-155DOI: (10.1016/S0022-5223(99)70480-0) Copyright © 1999 Mosby, Inc. Terms and Conditions

Fig. 6This is a stylized representation of the angle of tilt of the effective valvular orifice (ellipses) as estimated from the 23 specimens. The solid lines represent the cases with linear attachment; the ellipses with dotted lines and broken lines represent hearts with focal and hyphenated attachment of the anterosuperior leaflet, respectively. The vertical elipse represents the solitary case with the orifice parallel to the atrioventricular junction. The scale represents the maximal width of the orifice as a percentage of the distance between the atrioventricular junction and the apex of the right ventricle measured along the acute margin. The Journal of Thoracic and Cardiovascular Surgery 1999 117, 148-155DOI: (10.1016/S0022-5223(99)70480-0) Copyright © 1999 Mosby, Inc. Terms and Conditions