IPCCC: 07.10.01, 07.10.17, 12.14.02, 12.24.23
 

AEPC Derived Term:    

Perimembranous ventricular septal defect (VSD) (07.10.01)
Ventricular septal defect (VSD) + malaligned outlet septum anteriorly (07.10.17)
Pulmonary trunk band (PA band) (12.14.02)
Ligation of patent arterial duct (PDA)(12.24.23)

EACTS-STS Derived Term:    

VSD, Type 2 (Perimembranous) (Paramembranous) (07.10.01)
VSD-modifier for infundibular septal morphology, VSD + malaligned outlet septum, Anterior deviation of infundibular septum (07.10.17)
PA Banding, Band on main pulmonary artery (12.14.02)
PDA closure, Surgical therapy, Ligation (12.24.23)

Definition:  A ventricular septal defect contiguous with the site of the membranous septum. The membranous septum is defined as the area of the septum contiguous with the fibrous continuity between the leaflets of an atrioventricular valve and an arterial valve.
 

Commentary: Hearts with antero-cephalad malalignment of the muscular outlet, or conal, septum are usually found in the setting of tetralogy of Fallot. In the specimen shown here, however, the malaligment of the outlet septum did not cause sub-pulmonary obstruction. This arrangement, with an unobstructed outflow tract, is the lesion illustrated by Victor Eisenmenger at the end of the nineteenth century, and known by many as the “Eisenmenger VSD”. The very existence of this type of heart, with an unobstructed subpulmonary outlet, showed that something extra is needed over and above the anterocephalad deviation of the outlet septum so as to produce the phenotypic morphology of tetralogy of Fallot. The additional feature in tetralogy is an abnormal arrangement of the septoparietal trabeculations, which in most instances are additionally hypertrophied. It is the combination of the deviated outlet septum and the abnormal septoparietal trabeculations that produces the “squeeze” at the mouth of the subpulmonary infundibulum that is the essence of tetralogy of Fallot.
 

Modality: Anatomic specimen

Orientation: Anterior superior view

Description:  This view of the great vessels as they exit  the ventricular mass shows the aorta arising slightly to the right of its usual position. The pulmonary trunk is dilated with a pulmonary artery band in place and a ligated arterial duct. The right atrium is dilated and there is right ventricular hypertrophy.

Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label:   A071017-74a

Source of Image: Van Mierop Archive, University of Florida, Gainesville, FL

Image Certification: pending

AWG Rating:  pending

Modality: Anatomic specimen

Orientation:  Right ventricular view

Description:  Image of the same heart demonstrating on the anterior apical view of the right ventricle the antero-superior malalignment of the outlet septum into the right ventricle with an unobstructed subpulmonary outlet. The aorta overrides the ventricular septum, although the aortic valve is not appreciated in this view. This defect is consistent with an Eisenmenger type of ventricular septal defect.

Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label:    A071017-74b

Source of Image:  Van Mierop Archive, University of Florida, Gainesville, FL

Image Certification: pending

AWG Rating:  pending

Modality: Anatomic specimen

Orientation: Left ventricular view

Description:  In this anatomic view of the opened left ventricle, the free wall has been lifted away to demonstrate the aortic root. The aorta clearly overrides the interventricular septum with a good portion of the aortic valve supported in the right ventricle.

Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label:  A071017-74c

Source of Image: Van Mierop Archive, University of Florida, Gainesville, FL

Image Certification: pending

AWG Rating:  pending

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