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summARY A classification with clinical significance is proposed for ventricular septal defect based on
the study of 220 hearts with defects of the ventricular septum. All had atrioventricular and ventriculo-
arterial concordance with normal relations of cardiac structure. For the purpose of classification, the
ventricular septum was considered as possessing muscular and membranous portions, the muscular
septum itself being divided into inlet, trabecular, and outlet (or infundibular) components. Defects were
observed in the area of the membranous septum, termed perimembranous defects; within the muscular
septum, termed muscular defects; or in the area of septum subjacent to the arterial valves, termed
subarterial infundibular defects. Perimembranous defects were found extending either into the inlet,
trabecular, or infundibular septa. Muscular defects were found in or between the inlet septum,
trabecular septum, or infundibular septum. Review of the angiograms showed that the classification
was easy to use in the catheterisation laboratory, and our observations suggest that the precision thus
obtained has considerable surgical significance.
In this report, we present a simplified concept for sylvania. In all, 220 hearts were studied (Table).
the classification of ventricular septal defects based Only hearts with isolated or multiple ventricular
on the study of over 200 pathological specimens septal defects in the presence of atrioventricular
with atrioventricular concordance, ventriculoarterial and ventriculoarterial concordance were included.
concordance, and usual relations of intracardiac We excluded hearts with tetralogy of Fallot' but
structures. We have shown it to be useful for angio- included hearts with ventricular septal defect
graphic diagnosis and we believe it to have con- associated with valvar pulmonary stenosis. Some of
siderable surgical relevance. the hearts have previously been illustrated2 and an
earlier concept3 was based on study of others but the
Subjects and methods series as a whole has not been analysed previously.
The defects were studied with particular reference
The hearts studied were taken from the cardio- to their relation to the atrioventricular valves, the
pathological collections of the Cardiothoracic arterial valves, and the muscular bundles of the right
Institute, Brompton Hospital, London; the Royal ventricle, including the medial papillary muscle.
Liverpool Children's Hospital; the University of The defects will be described as viewed by the
Sheffield; Grimsby General Hospital; Welhelmina angiographer or clinician, with the patient con-
Gasthuis, Amsterdam; St Antonius Ziekenhuis, sidered as being in an upright position. When the
Utrecht: Department of Anatomy, Rijksuniversity, hearts have been photographed they have been
Leiden; the Mayo Clinic, Rochester, Minnesota; oriented as far as possible to achieve this positioning.
and the Children's Hospital of Pittsburgh, Penn- Photographs have been taken from the left ventricle,
* During the course of the work Benigno Soto was a Visiting Fellow
with the apex of the heart to the bottom of the frame
at the Cardiothoracic Institute. The work was supported by the and the aortic valve to the top.
Joseph Levy Foundation together with the British Heart Foundation, The angiographic feasibility of the suggested
and part was carried out while R H Anderson was in receipt of the classification was assessed by studying the angio-
Excerpta Medica Travel Award, 1977.
Received for publication 3 September 1979 grams from patients with ventricular septal defects
332
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Classification of VSD 333
and antrioventricular and ventriculoarterial con- trabeculae and bands observed in the normal right
cordance and usual relations investigated in the ventricle. The normal pulmonary and tricuspid
Department of Paediatric Cardiology, University of valves are separated by an extensive ledge termed in
Alabama in Birmingham, Birmingham, Alabama the normal heart the crista supraventricularis. It has
(courtesy of Dr L M Bargeron, Jr). Illustrative two components. Part of the normal crista addi-
examples of the classification were obtained from tionally separates the aortic and pulmonary valves
these patients. and is the outlet or infundibular septum. The other
part of the crista between the pulmonary and tri-
Results cuspid valves is the ventriculoinfundibular fold.4
The extensive septal trabeculation of the right
(1) CONSTITUENTS OF NORMAL ventricle is also considered to be part of the crista
VENTRICULAR SEPTUM AND TERMINOLOGY by some authors5 and is termed the 'septal band'.
OF MUSCLE BUNDLES Though yet others do not consider the septal band
For the purpose of classification of septal defects, to be part of the crista,6 in our opinion it is better
we have considered the ventricular septum as considered a separate structure and named the
having four components: the inlet septum, trabe- trabecula septomarginalis.4 It is a septal trabecula
cular septum and outlet or infundibular septum on the right aspect of the trabecular septum, forming
(together making up the muscular septum), and the the superficial stratum of the septum. An important
membranous septum (Fig. 1). The inlet septum small papillary muscle of the tricuspid valve arises
separates the septal cusps of the mitral and tricuspid from the posterior limb of the trabecula septo-
valves. It merges imperceptibly with the trabecular marginalis and supports the anteroseptal commis-
septum which is the largest part, extending out to sure of the tricuspid valve. It is the medial papillary
the ventricular apices, and separates the finely muscle. The apical part of the trabecula septo-
trabeculated left ventricular apex from the more marginalis is frequently enlarged and hypertro-
coarsely trabeculated right ventricular apical zone. phied, and may form a 'two-chambered right
It in turn merges imperceptibly with the outlet or ventricle'. This feature does not affect the classifica-
unfundibular septum which separates the right and tion of septal defects.
left ventricular outlet tracts, being considerably
more extensive on its right ventricular aspect than (2) CATEGORISATION OF VENTRICULAR
on the left. The membranous septum in the normal SEPTAL DEFECTS (Table)
heart is a small structure, divided into two parts by Our study has shown that on the basis of the septal
the insertion of the septal leaflet of the tricuspid division given above, isolated defects exist for the
valve. This insertion produces an atrioventricular most part in the area of the membranous septum
and an interventricular component, the sizes of (Fig. 2). However, as indicated by Becu et al.5 and
which vary in different hearts. endorsed by others, these defects additionally in-
In order to describe ventricular septal defects, it volve the area of muscular septum surrounding the
is also necessary to define the nature of muscular membranous septum itself, which may be present as
al) infundibular
b) trabecular
c) inlet
a remnant in the roof of such defects. Many have the inlet and trabecula septa (termed inlet muscular
indicated that 'membranous' is an inappropriate defects), within the trabecular septum itself (termed
term for such defects. We have taken note of these trabecular muscular defects), or between the in-
objections and have employed the term perimem-
branous to describe these defects. Perimembranous Table Categorisation of ventricular septal defects in
defect may involve the area of either the inlet, 220 hearts
trabecular, or infundibular septum contiguous with Type Subcategories No. studied
the area normally closed by the membranous septum Perimembranous (a) Inlet 55
(Fig. 3). (b) Trabecular 56
In contrast to perimembranous defects, in which (c) Infundibular 42
part of the rim of the defect is always formed by part Muscular (a) Posterior (inlet) 15
of the central fibrous body, other defects have purely (b) Trabecular 40
(c) Infundibular 3
muscular rims (Fig. 2). They are collectively termed Subarterial infundibular 12
muscular defects, and may exist in the area between Mixed defects 3
PERIMEMBRANOUS VSD
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Classification of VSD 335
fundibular and trabecular septa (termed infundi- primarily into the inlet septum, the trabecular
bular muscular defects). A further type of defect septum, or the infundibular septum.
exists in the area normally formed by the infundi- (a) Defects extending into inlet septum When
bular septum which does not have a purely muscular viewed from the right ventricular aspect (Fig. 4A),
rim, and is therefore not a muscular defect, but these defects were beneath the septal cusp of the
equally is not a perimembranous defect. The non- tricuspid valve. Their atrial margin was the area of
muscular rim of these defects is formed by the con- tricuspid-mitral continuity. The floor and anterior
tiguous arterial valves and they are termed sub- margin of the defect was the crest of the inlet and
arterial infundibular defects (Fig.2). trabecular septa which ran up in the roof to merge
with the infundibular septum. This septum was
I: Perimembranous defects normally positioned and fused with the trabecular
The unifying feature of these defects was that all septum as in the normal heart. The medial papillary
had the area of tricuspid-mitral-aortic fibrous con- muscle was usually found above the defect. From
tinuity (central fibrous body) as part of their rim the left ventricular aspect it was seen that the pos-
(Fig. 3). The precise boundaries of the defects, and terior margin of the defect was an extensive area
their relation to the aortic and atrioventricular of aortic-mitral-tricuspid continuity. The non-
valves, depended on whether the defect extended coronary cusp was in extensive continuity with,the
Fig. 4 Figures of perimembranous defects extending predominantly into the inlet septum (Fig. 4A); the trabecular
septum (Fig. 4B) and the infundibular septum (Fig. 4C). The inlet defect is shown in close up. It lies beneath the septal
leaflet (SL) of the tricuspid valve. Medial papillary muscle (MPM) and the anterosuperior leaflet (ASL) are above
the defect. FO, fossa ovalis; CS, coronary sinus. The trabecular defect (D) is behind the tricuspid valve (TV)
extending between the limbs of the trabecula septomnarginalis. The infundibular defect has some degree of override so
that the aortic cusps are visible.
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336 Soto, Becker, Moulaert, Lie, Anderson
tricuspid valve, making the aortic valve cusps the was beneath the septal leaflet of the tricuspid valve,
roof of much of the defect (Fig. 5A). In four hearts and its atrial border was a small area of aortic-
the septum was 'scooped' out up to the crux cordis mitral-tricuspid continuity (Fig. 4B). Its floor was
as is usually found in atrioventricular defects (atrio- the inlet septum, merging anteriorly with the
ventricular canal malformations or endocardial trabecular septum buttressed by the trabecula
cushion defects) (Fig. 6) and in two of these hearts septomarginalis. The roof of the defect was the in-
there was a cleft of the mitral valve. The roof of the fundibular septum, which was normally aligned
defect in all these hearts was the infundibular relative to the trabecular septum so that no aortic
septum which separated the right coronary aortic overriding was 'present (Fig. 4B). The medial
cusp from the edge of the defect. There was no papillary muscle tended to take origin from the mid-
malalignment of the septal structures in these point of the right side of the defect, and was at-
hearts. tached to the septal commissure of the tricuspid
valve across the defect. This relation was better seen
(b) Defects extending into trabecular septum In from the left (Fig. 5B), this view also showing well
these specimens the defect was more elongated and the primary extension into the trabecular septum.
extended towards the ventricular apex. When The long axis of the defect was from the aortic valve
viewed from the right ventricle, its posterior rim towards the left ventricular apex. In many hearts
Fig. 5 The same three defects illustrated in Fig. 4 viewed from the left ventricular aspect. Fig. 5A shows the inlet
defect. The inlet septum (IS) is deficient. RCC, right coronary cusp of aortic valve; NCC, non-coronary cusp;
MV, mitral valve. Fig. 5B shows the trabecular defect. The inlet septum is better formed. The defect points toward the
apex and a remnant of the membranous septum (Rem MS) separates the defect from the aortic valve. Inf S,
infundibular septum. Note the different orientation of the infundibular defect shown in Fig. 5C.
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Classification of VSD 337
AMIL~~~~
A
Fig. 8 A trabecular septal defect viewed from the right ventricle (A), and the left ventricle (B).
:iAo' Br Heart J: first published as 10.1136/hrt.43.3.332 on 1 March 1980. Downloaded from http://heart.bmj.com/ on August 16, 2023 by guest. Protected by copyright.
Classification of VSD 339
Fig. 9 A muscular defect of the infundibular septum viewed from the right ventricle (A), and the left ventricle (B).
Note that the posterior limb of the trabecula septomarginalis (arrowed) fuses with the ventriculoinfundibular fold
(VIF) in the posterior rim of the defect. The infundibular septum separates the defect from the pulmonary valve.
The membranous septum is intact (abbreviations as before).
III: Subarterial infundibular defects were free to move into the right ventricular outflow
These defects were similar to the muscular infundi- tract, and some of the hearts had aortic cusp pro-
bular defects except that the infundibular septum lapse. In the floor of the defect, as in the muscular
was totally deficient, so that the aortic and pul- infundibular defects, the posterior limb of the
monary valves were contiguous in the roof of the trabecula septomarginalis extended to the ven-
defect (compare Fig. 9 and 10). Because of this, the triculoinfundibular fold, forming a muscular rim in
left coronary and right coronary aortic valve cusps front of a normally formed membranous septum
I 'Cl'.. M
X .~~~~~~~~~~~~~
Fig. 11 Angiographic feature of perimembranous ventricular septal defects seen in four chamber' views. The
diagnoses were subsequently verified at operation. (A) Perimembranous ventricular septal defect excavated in the inlet
septum. The ventricular septal defect is large, roofed by the aortic valve. The inferior border is near the crux of the
heart (arrow-head). A large segment of the septal tricuspid leaflet (arrow) is in contact with the defect, but both
atrioventricular valves are well formed. AO, aorta; PA, pulmonary artery; LV, left ventricle. (B) Perimembranous
defect excavated in the trabecular septum. The defect is roofed by the aortic valve (non-coronary cusp) and its
anterior border is the upper portion of the trabecular septum (arrow). Notice that the contrast media from the left
ventricle (LV) opacifies the right ventricle (RV) and also the right atrium (RA). The left ventricle-right atrium
connection is through the medial commissure of the tricuspid valve which covers the ventricular septal defect.
AO, aorta; PA, pulmonary artery. (C) Perimembranous defect excavated in the infundibular septum. The defect is
located immediately beneath the right and non-coronary (aortic) cusps (arrows). The contrast media injected into the
left ventricle (LV) passes into the right ventricle (RV) opacifying the trabeculated portion mainly. The infundibular
septum (IS) is deviated anteriorly, leaving the right and non-coronary cusps above the right ventricle.
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Classification of VSD 341
Fig. 12 Angiographic feature of muscular ventricular septal defects seen in four chamber' views. The diagnosis in
each case was verified at surgery. (A) Muscular inlet. The defect (arrows) is located in the inlet portion of the ventricular
septum discontinuous from the mitral and tricuspid annuli. RV, right ventricle; LV, left ventricle. (B) Muscular
trabecular. The defect (arrow) is located in the trabecular portion of the septum near its posterior border. R V, right
ventricle; LV, left ventricle; AO, aorta; PA, pulmonary artery. (C) Muscular infundibular. The defect (arrow) is in
the right border of the left ventricular outflow tract, separated from the arterial and atrioventricular valves. Note
the early opacification of pulmonary artery (PA). AO, aorta; RV, right ventricle; LV, left ventricle.
siderable value in surgical viewpoint. The main either the right atrium (Fig. 14) or a right ventri-
thrust of the surgical classification of Kirklin and cular infundibulotomy). In contrast, a muscular
his colleagues1' 12 was to distinguish those defects in defect in the inlet septum will have the conduction
relation to the aortic valve ('high' defects) from tissue related to its superoanterior quadrant (to the
those not in relation to the aortic valve ('low' surgeon's left hand as viewed from atrium (Fig. 14)
defects). Our classification refines this possibility, or infundibulum). Muscular defects in the trabe-
since it distinguishes defects with entirely muscular cular septum are unlikely to be related to the non-
rims, which clearly are never directly related to the branching or branching components of the conduc-
aortic valve, from those with rims formed by in- tion tissue axis, though they may be related to
trinsically fibrous tissue in which the aortic valve bundle-branches,"7 while infundibular, muscular,
forms part of this rim. It further distinguishes the or subarterial defects are unrelated to the conduc-
latter group into those in which the aortic valve is tion tissue.18 The recognition of a perimembranous
related to the defect as part of the central fibrous defect as inlet, trabecular, or infundibular also
body (perimembranous defects) from those in provides information regarding the direct relation
which it is related to the defect in continuity with of the ventricular conduction tissue axis to the
the aortic and pulmonary valves (subarterial in- septal rim.'8
fundibular defects). However, the surgical value of In addition to providing this information which
the present classification goes far beyond the relation we believe to be of value to both diagnostician and
of the defect to the aortic valve. The recognition of surgeon, our study helped to clarify several points
a defect as perimembranous, muscular, or infundi- which previously had given us some problems.
bular immediately alerts the surgeon to the likely Firstly, it became evident that though most defects
disposition of the conduction tissues. If a defect is were in the region of the membranous septum, they
perimembranous, previous studies'5-'7 have shown represented more than mere absence of the mem-
that the atrioventricular conduction axis will always branous septum. Becu et al.5 have previously em-
be related to the posteroinferior quadrant of the phasised this point. Goor and his colleagues'4 la
defect (to the surgeon's right hand as viewed from similarly recognised that most defects in this area
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342 Soto, Becker, Moulaert, Lie, Anderson