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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.

Br HeartJ 1980; 43: 332-343

Classification of ventricular septal defects*


BENIGNO SOTO, ANTON E BECKER, ANDRE J MOULAERT, J T LIE,
ROBERT H ANDERSON
From the Departments of Radiology, University of Alabama in Birmingham, Birmingham, Alabama, USA;
Pathology, Wilhelmina Gasthuis, University of Amsterdam, The Netherlands; Paediatric Cardiology,
Wilhelmina Kinderziekenhuis, University of Utrecht, The Netherlands; Pathology, Mayo Clinic,
Rochester, Minnesota, USA, and Paediatrics, Cardiothoracic Institute, Brompton Hospital, University of
London, UK

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

Fig. 1 Diagram illustrating the


different muscular components of
the ventricular septum and their
conjunction with the membranous
septum.

a) Right Ventricle b ) Lef t Ventr-ic le


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334 Soto, Becker, Moulaert, Lie, Anderson
Defects with partly fibrous rims

Fig. 2 Diagram illustrating the


proposed classification of
ventricular septal defects.
2' < < > M uscular defects

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

Medial Papillary Muscle


Remnant of Membranous Septum may be multiple and
frequently presents may arise from
I,Infundibular Septum

Fig. 3 Diagram illustrating the


types of perimembranous septal
defect. TSM, trabecula
septomarginalis; Infund,
infundibular.

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

remnants of tissue were delaminated from the


central fibrous body in the roof of the defect, as _
viewed from the left. The aortic valve noncoronary
cusp was in continuity with the mitral valve, but the
membranous septal remnant separated the aortic
valve from the tricuspid valve (Fig. 5B). The M PM
anterior margin of the roof of the defects when
viewed from the left ventricle (Fig. 5B) was the
infundibular septum, which formed a muscular bar
separating the right coronary cusp from the defect.
In these defects, therefore, a much smaller area of
the aortic valve was directly contiguous with the i _
defect as compared with the perimembranous inlet .......3
defect (compare Fig. 5A and 5B).
(c) Defects extending into the infundibulum The in-
fundibular septum of these hearts was less well
formed than in the other perimembranous defects A

AMIL~~~~

Fig. 7 Photographs illustrating a muscular defect


between the inlet septum and the trabecular septum.
Fig. 7A is a right ventricular view, 7B a left ventricular
~~~~~~~~~~iew(abbreviations as before).

and was usually raised to the right so that the aortic


valve overrode the right ventricle (compare Fig. 4B
and 4G). The atrial edge of the defect as viewed
from the right ventricle was again an area of tri-
cuspid-mitral continuity, and this became con-
tinuous anteriorly with the overriding aortic valve
which formed the roof of the defect. The infundi-
bular septum formed the anterior edge of the roof
merging with the trabecular septum and trabecula
septomarginalis in the anteroinferior rim of the
defect. The medial papillary muscle was below the
defect (Fig. 4C), but accessory papillary muscles
were occasionally observed arising from the in-
fundibular septum. The anterior extension of the
Fig. 6 Photograph of an inlet defect (of atrioventricular defect was well seen from the left ventricular aspect
canal type). The inlet septum is deficient right to the (Fig. 5G). Its long axis, in contrast to the trabecular
crux of the heart. Fig. 64 shows the left ventricular perimembranous defect, was oriented from left to
aspect and 6B the right ventricular aspect. There was no right (compare Fig. 5B and 5C). The posterior
cleft in the mitral valve (abbreviations as before). margin of the defect, as viewed from the left
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338 Soto, Becker, Moulaert, Lie, Anderson
ventricle, was the central fibrous body. In the the left than the right side. The openings as seen
posterior margin of the roof, the aortic valve from the right ventricle were closely related to the
noncoronary cusp was directly contiguous with the edges of the trabecula septomarginalis (Fig. 8A).
defect as a consequence of the aortic overriding, but From the left it was seen that the defects were
in the anterior margin the valve was separated from within the trabecular septum (Fig. 8B) but their
the defect by the infundibular septum (Fig. 5G). position was variable. The membranous septum
was normally formed in these hearts.
II: Muscular defects
(a) Defects in inlet area of muscular septum These (c) Defects in infundibular area of septum Three
defects were beneath the septal cusp of the tricuspid specimens were encountered in which a defect with
valve (Fig. 7A), but possessed completely muscular entirely muscular rims was located between the
rims. When viewed from the left ventricle, a rim of ventricular outflow tracts. As viewed from the right
inlet septum was present beneath and between the ventricle, the roof of the defect was the remnant of
septal cusps of the tricuspid and mitral valves the infundibular septum. This merged anteriorly
(Fig. 7B). This rim crossed obliquely the crest of with the anterior limb of the trabecula septo-
the trabecular septum. It extended from the right marginalis (Fig. 9A) which formed the floor of the
ventricle, where it was fused with the trabecula defect. Posterior, the infundibular septum merged
septomarginalis posteriorly into the left ventricle, with the ventriculoinfundibular fold and the
where it fused with the trabecular septum. Fre- posterior limb of the trabecula septomarginalis.
quently this latter fusion point was overlaid by a This fusion of muscular structures formed a mus-
prominent posterior trabecula. The size of the cular rim in front of a normally formed and posi-
defect varied according to the degree of develop- tioned membranous septum. When viewed from the
ment of the trabecular septum. A membranous left ventricle, the defect was seen to be completely
septum was present and intact in these hearts, separated from the aortic valve by the infundibular
having both atrioventricular and interventricular septum. Posterior to this the aortic valve was con-
components. tinuous with the central fibrous body (Fig. 9B). The
difference between this defect and the perimem-
(b) Defects in the trabecular area of the muscular branous infundibular defect was that the aortic
septum These defects in the muscular septum were valve did not form part of the rim of the defect
frequently multiple and better appreciated from (compare Fig. 5C and 9B).

A
Fig. 8 A trabecular septal defect viewed from the right ventricle (A), and the left ventricle (B).
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Classification of VSD 339

n~~~~~~ -.:i.: :.'..7

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

Fig. 10 Right ventricular (A)


and left ventricular (B) views to
a subarterial septal defect. The
posterior limb (PL) of the
- trabecula septomarginalis fuses
gj>Ww with the ventriculoinfundibular
MV fold separating
the normally the defect
formed from
membranous
septum. Deficiency of the
infundibular septum permits
pulmonary to aortic fibrous
j continuity, P-AoC, Ao-PC.
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340 Soto, Becker, Moulaert, Lie, Anderson
(Fig. 10). When viewed from the left ventricle, the Discussion
right and left coronary cusps of the aortic valve
formed the entire roof of the defect, being con- The classification of isolated ventricular septal de-
tiguous with the pulmonary valve and distinguishing fect here presented is an eclectic modification of
the defects from muscular infundibular defects categorisations of earlier workers5 9-14 together with
(compare Fig. 9B and lOB). our own earlier attempts to achieve a satisfactory
nosology.2 3 In the present categorisation we have
ANGIOGRAPHIC CORRELATIONS used purely descriptive terms to describe the three
Examination of angiograms from patients studied components of the muscular septum delineated on
at the University of Alabama in Birmingham developmental grounds by Goor et al.,'4 and we
(courtesy of Dr L M Bargeron Jr) showed that using believe that this usage has increased the value of the
projections designed to profile the ventricular categorisation. Certainly by using angiographic
septum7 it was possible to distinguish perimem- views designed to profile the ventricular septum7 it
branous (Fig. 11) from muscular (Fig. 12) and has proved feasible to distinguish defects existing
subarterial infundibular (Fig. 13) defects.8 Further- in relation to these three parts of the muscular
more, careful assessment of each type showed that it septum in addition to distinguishing between
was possible to distinguish the three varieties of perimembranous, muscular, and subarterial defects.
both perimembranous (Fig. 1 lA-C) and muscular Although it clearly has value in angiographic
(Fig. 12A-C) defects. diagnosis, it is our contention that it has con-

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

Fig. 13 Angiographic appearance


of subarterial ventricular septal
defect, left ventriculogram in (A)
'long axial', and (B) elongated
right anterior oblique views. The
defect is located in the infundibular
septum roofed by the arterial
valves seen in both projections.
The atrioventricular valves are
not related with the ventricular
septal defect. AO, aorta; PA,
pulmonary artery; RV, right
ventricle; LV, left ventricle.

were not 'true' defects of the membranous septum,


but they did recognise a small group which they PERIMEMBRANOUS DEFECT
categorised as membranous defects. In our series, Aortic atrioventricular continuity
we did not encounter any defects so small that we forms part of defect rim
-bundle to right hand as
considered them truly to be mere absence of the seen from atrium
interventricular membranous septum. Instead, it \ ni:rI:T n
was our interpretation that all defects resulted from
deficiency of the muscular septum in the environs
of the interventricular membranous septum, which
was frequently present as part of the border of the
defects. Hence, our suggestion of the term 'peri-
membranous' to describe these defects, and our
subdivision of the group depending on whether the
muscular deficiency affected primarily the inlet,
trabecular, or outlet components of the muscular
septum. It is possible that tiny defects could result
simply from absence of the interventricular mem-
branous septum, or that alternatively a small per-
foration could be found in an aneurysmal mem-
branous septum which has closed an existing defect.
If required, the term 'membranous septal defect'
could accurately be applied to such lesions.
The second point relates to the so-called 'isolated N.B.
ventricular septal defect of persistent common The medial papillary muscle complex
atrioventricular type'.20 As one of us has indicated, is variable :- it is not a good guide
most of these defects are really defects of the inlet to the right bundle branch in isolated VSD
septum extending into the area of the membranous Fig. 14 Diagrammatic representation of the different
septum, and have presently been categorised as relations of perimembranous and muscular inlet defects
perimembranous inlet defects.2 It was significant to the atrioventricular conduction tissue axis as viewed
that not all the perimembranous inlet defects we by the surgeon from the right atrium.
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 343
studied exhibited the stigmata of atrioventricular 'Becu LM, Fontana RS, DuShane JW, Kirklin JW,
defects, namely disproportion between the inlet Burchell HB, Edwards JE. Anatomic and pathologic
and outlet dimensions of the muscular septum and studies in ventricular septal defect. Circulation 1956;
malorientation of the aortic valve. Indeed, not all 14: 349-64.
the defects illustrated by Neufeld et al.20 showed 6Van Praagh R. What is the Taussig-Bing malforma-
tion? Circulation 1968; 38: 445-9.
these features. Only four of the 55 inlet perimem- 'Bargeron LM Jr, Elliott LP, Soto B, Bream PR,
branous defects we examined exhibited the Curry GC. Axial angiocardiography in congenital
'scooped-out' septum so typical of the atrioven- heart disease. Section I. Concept, technical and
tricular defect, two of these having clefts in their anatomic considerations. Circulation 1977; 56: 1075-
mitral valves. If the term 'isolated atrioventricular 83.
canal defect' is to be used, it is our preference to 8Soto B, Coghlan C, Bargeron LM Jr. Angiography of
restrict it to these hearts, while still preferring the the ventricular septal defects. In: Anderson RH,
description 'perimembranous inlet defect with gross Shinebourne EA, eds. Paediatric cardiology 1977.
deficiency of the inlet septum' as being less am- Edinburgh: Churchill Livingstone, 1978: 125-37.
9Rokitansky C von. Die Defecte der Scheidewdnde des
biguous. Herzens. Wien: Wilhelm Braumuller, 1875.
Thus, we have presented an eclectic classification 'Warden HE, DeWall RA, Cohen M, Varco RL,
of ventricular septal defects based on the premise Lillehei CW. A surgical-pathologic classification for
that the ventricular septum has membranous and isolated ventricular septal defects and for those in
muscular portions, the latter itself having inlet, Fallot's tetralogy based on observations made on 120
trabecular, and outlet components. We have shown patients during repair under direct vision. J Thorac
that the classification, based on study of patho- Surg 1957; 33: 21-44.
logical material, is of value in the angiographic "Kirklin JW, McGoon DC, DuShane JW. Surgical
treatment of ventricular septal defect. J Thorac
laboratory, and that the diagnostic precision thus Cardiovasc Surg 1960; 40: 763-75.
afforded has considerable surgical significance. We 1Kirklin JW, Harshbarger HG, Donald DE, Edwards
offer the classification as a simple alternative to those JE. Surgical correction of ventricular septal defect:
presently available. anatomic and technical considerations. J Thorac Surg
1957; 33: 45-59.
We are indebted to our colleagues who permitted us 1Lev M. The pathologic anatomy of ventricular septal
to study hearts from their collections, namely, defect. Dis Chest 1959; 35: 533-45.
Professor F J Macartney, The Hospital for Sick "Goor DA, Lillihei CW, Rees R, Edwards JE. Isolated
Children, London, UK; Dr J L Wilkinson, Royal ventricular septal defect; development basis for various
Liverpool Children's Hospital, UK; Dr L M Gerlis, types and presentation of classification. Chest 1970;
Grimsby General Hospital, UK; Professor J Emery, 58: 468-82.
Sheffield University, UK; Dr A Oppenheimer- "Truex RC, Bishof JK. Conduction system in human
hearts with interventricular septal defects. J Thorac
Decker, Leiden, The Netherlands; Dr J Van Gorp, Surg 1958; 35: 421-39.
Utrecht, The Netherlands, and Dr J R Zuberbuhler, 16Lev M. The architecture of the conduction system in
Pittsburgh, Pa, USA. We thank Dr J W Kirklin, congenital heart disease. III. Ventricular septal defect.
Birmingham, Alabama, USA, for his advice and Arch Pathol 1960; 70: 529-49.
critical appraisal of the manuscript, and Dr L M "Latham RA, Anderson RH. Anatomical variations in
Bargeron Jr, Birmingham, Alabama, USA, for atrioventricular conduction system with reference to
permission to study and publish the angiograms of ventricular septal defects. Br HeartJ 1972; 34: 185-90.
his patients. '8Milo S, Ho SY, Wilkinson JL, Anderson RH. The
surgical anatomy and atrioventricular conduction
References tissues of hearts with isolated ventricular septal
defects. 7 Thorac Cardiovasc Surg 1980; in the press.
'Becker AE, Conner M, Anderson RH. Tetralogy of '9Goor DA, Lillehei CW. Congenital malformations of
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2Moulaert A. Anatomy of ventricular septal defect. In: 2"Neufeld HN, Titus JL, DuShane JW, Burchell HB,
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4Anderson RH, Becker AE, Van Mierop LHS. What stitute, Brompton Hospital, Fulham Road, London
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