CONGENITAL HEART DISEASE: FONTAN

Anatomy of the ventricular septal defect in outflow tract defects:

Similarities and differences
Meriem Mostefa-Kara, MD,a,d Damien Bonnet, MD, PhD,a,b,c Emre Belli, MD,d Elie Fadel, MD, PhD,e and
Lucile Houyel, MDd

ABSTRACT
Objective: The study objective was to analyze the anatomy of the ventricular
septal defect found in various phenotypes of outflow tract defects.
Methods: We reviewed 277 heart specimens with isolated outlet ventricular
CHD

septal defect without subpulmonary stenosis (isolated outlet ventricular septal

defect, 19); tetralogy of Fallot (71); tetralogy of Fallot with pulmonary atresia
(51); common arterial trunk (54); double outlet right ventricle (65) with subaortic,
doubly committed, or subpulmonary ventricular septal defect; and interrupted
aortic arch type B (17). Special attention was paid to the rims of the ventricular An outlet VSD viewed from the right ventricle, located between the 2
septal defect viewed from the right ventricular side and the relationships between limbs of the septal band.
the tricuspid and aortic valves.
Central Message
Results: The ventricular septal defect was always located in the outlet of the right The VSD in outflow tract defects is always an outlet VSD,
ventricle, between the 2 limbs of the septal band. There was a fibrous continuity cradled between the 2 limbs of the septal band. Differ-
between the tricuspid and aortic valves in 74% of specimens with isolated outlet ences between these defects regarding the presence of aor-
totricuspid continuity suggest an anatomic continuum,
ventricular septal defect, 66% of specimens with tetralogy of Fallot, 39% of spec- related to various degrees of abnormal rotation of the
imens with tetralogy of Fallot with pulmonary atresia, 4.6% of specimens with outflow tract during the heart development.

double outlet right ventricle, 1.8% of specimens with common arterial trunk,
and zero of specimens with interrupted aortic arch type B (P<.005). When pre- Author Perspective
sent, this continuity always involved the anterior tricuspid leaflet. This anatomic study offers a new perspective on the group
of outflow tract defects. The cornerstone of these defects is
the outlet VSD, which always opens in the outflow tract of
Conclusions: The ventricular septal defect in outflow tract defects is always an the right ventricle between the 2 limbs of the septal band,
outlet ventricular septal defect, cradled between the 2 limbs of the septal band. irrespective of the presence or absence of a fibrous conti-
However, there are some differences regarding the posteroinferior and superior nuity between the aortic and the tricuspid valves. This
anatomic approach underlines the anatomic difference be-
rims of the ventricular septal defect. These differences suggest an anatomic tween outlet VSD and isolated central perimembranous
continuum from the isolated outlet ventricular septal defect to the interrupted VSD. By reinforcing the hypothesis of an embryologic
origin common to all outflow tract defects, it might help
aortic arch type B rather than distinct physiologic phenotypes, related to various us to clarify the classification and terminology of VSD.
degrees of abnormal rotation of the outflow tract during heart development: min-
imal in isolated outlet ventricular septal defect; incomplete in tetralogy of Fallot,
tetralogy of Fallot with pulmonary atresia, and double outlet right ventricle; ab-
sent in common arterial trunk; and excessive in interrupted aortic arch type B.
(J Thorac Cardiovasc Surg 2015;149:682-8)

the outflow tract during cardiac development.1 Cardiac

Supplemental material is available online. neural crest defects are due to a defect of migration of
cardiac neural crest cells, leading to a lack of elongation
of the outflow tract and eventually to an abnormal
Outflow tract defects including cardiac neural crest defects development of the outlet part of the ventricular septum.2
(so-called conotruncal defects) and transposition of the This common embryologic origin expresses itself within
great arteries (TGA) are due to an abnormal formation of different anatomic phenotypes, including common arterial

From the Universit
e Paris Descartes,a Sorbonne Paris Cit
e, Paris, Imagine Institute, Funded by a grant from the French Society of Cardiology.
H^opital Necker enfants Malades, Paris, France; Paediatric Cardiology,b Centre Received for publication May 27, 2014; revisions received Nov 17, 2014; accepted
de R
ef
erence Malformations Cardiaques Cong
enitales Complexes - M3C, Necker for publication Nov 29, 2014; available ahead of print Feb 20, 2015.
Hospital for Sick Children, Assistance Publique des Hopitaux de Paris, Paris, Address for reprints: Meriem Mostefa-Kara, MD, 149 rue de Sevres, 75015 Paris,
France; Universit
e Paris Descartes,c Sorbonne Paris Cit
e, Paris, France; Congenital France (E-mail: meriem.kara@gmail.com).
Cardiac Surgery,d H^ opital Marie-Lannelongue, CMR-M3C, Universit
e Paris-Sud, 0022-5223/$36.00
Le Plessis-Robinson, France; and Laboratory of Surgical Research,e H^opital Copyright Ó 2015 by The American Association for Thoracic Surgery
Marie-Lannelongue, Le Plessis-Robinson, France. http://dx.doi.org/10.1016/j.jtcvs.2014.11.087

682 The Journal of Thoracic and Cardiovascular Surgery c March 2015

Mostefa-Kara et al Congenital Heart Disease: Fontan

analyzed 15 hearts with dilated or restrictive cardiomyopathy without

any associated congenital heart defects as controls.
Abbreviations and Acronyms The intracardiac anatomy of each heart specimen was studied with
CAT ¼ common arterial trunk particular attention paid to the VSD. The exact localization of the VSD
DORV ¼ double outlet right ventricle and its borders were carefully described viewed from the right ventricular
IAA ¼ interrupted aortic arch side, as well as the relationship between the tricuspid and aortic valves,
PA ¼ pulmonary atresia looking for a fibrous continuity between these 2 valves, and particularly
which tricuspid leaflet was involved in this continuity.
TGA ¼ transposition of the great arteries We chose to define the curved surface of the VSD by the surface that will
TOF ¼ tetralogy of Fallot be closed by the surgeon.9 This surface is different depending on whether
VSD ¼ ventricular septal defect each arterial trunk arises from its own ventricle or both arterial trunks arise
from the same ventricle as in DORV. In DORV, the VSD, roofed by the
outlet septum or its fibrous remnant, is different from the interventricular
trunk (CAT); tetralogy of Fallot (TOF) with or without communication that lies in the plane of the ventricular septum and is roofed

CHD
pulmonary atresia (PA) or absent pulmonary valve; double by the inner curvature of the heart.
The morphologically normal hearts were used as controls to analyze
outlet right ventricle (DORV) with subaortic, doubly
precisely the anatomy of the membranous septum and its relations with
committed, or subpulmonary ventricular septal defect the limbs of the septal band or septomarginal trabeculation. We also looked
(VSD); some forms of interruption of the aortic arch; and for the presence or absence of a demonstrable outlet septum and its
isolated VSD opening in the outlet of the right ventricle, orientation when the outlet septum was deviated anteriorly (into the right
with outlet septal malalignment.3 From a purely anatomic ventricle) or posteriorly (into the left ventricle) relative to the axis of the
remainder muscular ventricular septum.
standpoint, these congenital heart defects are described as
different, but all share the presence of a VSD, as described
by several authors.4-8 Embryologically, this VSD is Normal Anatomy of the Right Ventricle
To fully describe the morphologic features of the VSD, we have to
secondary to a lack of fusion between the outlet septum clearly define the terminology used to characterize the different parts of
and the septal band or septomarginal trabeculation the right ventricle (Figure 1).
because of an incomplete rotation of the outflow tract.3,9 The ventriculo-infundibular fold (grouping together parietal band and
The aim of this study was to analyze the anatomy of the subpulmonary conus) is a muscular band that separates the anterior leaflet
of the tricuspid valve from the leaflets of the pulmonary valve in the normal
VSD found in the various phenotypes of outflow
heart.17-19 The left extremity of the ventriculo-infundibular fold represents
tract defects on heart specimens and to determine its the upper part of the ventricular septum: the outlet (or conal) septum, which
localization, its anatomic characteristics, and the is fused with the upper extremity of the septal band (or septomarginal
rims and relationships to adjacent structures to search trabeculation), between its 2 limbs, anterosuperior and posteroinferior. In
for similarities or differences among these various the normal heart, the posteroinferior limb of the septal band, which carries
the papillary muscle of the conus and its attachments, is not fused with the
phenotypes.
ventriculo-infundibular fold but is separated from it by the atrioventricular
part of the membranous septum, connecting the anterior leaflet of the
MATERIAL AND METHODS tricuspid valve with the leaflets of the aortic valve.20 The interventricular
We analyzed the anatomy of the VSD in human hearts with outflow part of the membranous septum connects the septal leaflet of the tricuspid
tract defects using the heart specimens of the anatomic collection of valve with the right coronary and the noncoronary leaflets of the aortic
the French Reference Center for Complex Congenital Heart Defects valve (Figure 2). This will help us to analyze the localization of the VSD
(M3C) in the Marie-Lannelongue Hospital. This collection includes in the specimens and to describe precisely the rims of the VSD.
1366 heart specimens fixed in 10% formalin. We studied 277 heart
specimens with outflow tract defects, including 19 isolated VSDs open- Statistical Analysis
ing into the outlet of the right ventricle with overriding aorta but no StatView software (SAS Institute Inc, Cary, NC) was used for data
subpulmonary stenosis (isolated outlet VSD, including malalignment analysis. The qualitative anatomic variables were presented with
and doubly committed phenotypes); 71 with TOF (8 of them with ab- percentages. A chi-square test analysis was used to evaluate the possible
sent pulmonary valve); 51 with TOF-PA; 54 with CAT; 65 with DORV differences between the parameters.
(including 36 with subaortic VSD, 4 with doubly committed VSD, and
25 with subpulmonary VSD); and 17 with interrupted aortic arch
(IAA) type B. RESULTS
We chose to exclude hearts with DORV with noncommitted VSD, Localization of the Ventricular Septal Defect
because these defects are supposed to be related with distinct
All heart specimens, except 1 with an IAA type B, had a
embryologic mechanisms.10 We also excluded IAA type A because
this defect is generally thought to be an extreme form of aortic VSD. This VSD was, as expected, always located in the
coarctation.11 There was no IAA type C in the collection. Although outlet of the right ventricle, between the 2 limbs of the
TGA is also an outflow tract defect, we chose to study 104 hearts septal band (outlet-type VSD) (Figure 3). However, we
with TGA and associated VSD separately because several epidemio- could distinguish some anatomic differences within these
logic, experimental, and genetic studies tend to demonstrate that the outlet VSDs, depending on the rims of the VSD and more
morphogenesis of TGA could involve in part the laterality genes, such
as ZIC3, CFC1, and NODAL,12-14 leading to a possible disturbance of precisely on the presence or absence of a fibrous continuity
the ‘‘rotational’’ aspect of wedging15 and to the fusion of the outflow between the leaflets of the aortic (or truncal) and tricuspid
tract cushions in a parallel and not spiraling fashion.16 In addition, we valves, and on the presence or absence of an outlet septum.

The Journal of Thoracic and Cardiovascular Surgery c Volume 149, Number 3 683
 Congenital Heart Disease: Fontan Mostefa-Kara et al
CHD

FIGURE 1. Right ventricular aspect of the ventricular septum in a

normal heart. The muscular bands of the right ventricle are the
ventriculo-infundibular fold, including the parietal band (1) and
FIGURE 2. Localization of the membranous septum in the normal
subpulmonary conus (2); the outlet septum (*); the septal band or
heart viewed from the right ventricle. The membranous septum
septomarginal trabeculation with its 2 limbs, anterosuperior and
(transilluminated) ensures a fibrous continuity between the anteroseptal
posteroinferior (yellow Y); the moderator band; and the anterior papillary
commissure of the tricuspid valve and the aortic valve. The posteroinferior
muscle of the tricuspid valve. MB, Moderator band; PV, pulmonary valve;
limb of the septal band is separated from the ventriculo-infundibular fold
TV, tricuspid valve; VIF, ventriculo-infundibular fold; SB, septal band.
by the membranous septum. PV, Pulmonary valve; SB, septal band;
TV, tricuspid valve; VIF, ventriculo-infundibular fold.

Rims of the Ventricular Septal Defect

This anatomic characteristic could be analyzed in all the atrioventricular part of the membranous septum,
specimens except 2 with TOF-PA, because previous surgery connecting the anterior leaflet of the tricuspid valve and
made the analysis of the rims of the VSD difficult. All VSDs the aortic leaflets (Figure 2).
were located in the outlet of the right ventricle, between the In the hearts without fibrous continuity between the
2 limbs of the septal band, but there were differences aortic (or truncal) and tricuspid valves, the posteroinferior
between them regarding the nature of their rims and more rim of the VSD was entirely muscular because of the fusion
particularly of the posteroinferior rim. We identified 2 of the posteroinferior limb of the septal band with the
different types: one with a fibrous continuity between the ventriculo-infundibular fold (Figure 4).
aortic (or truncal) valve and the tricuspid valve in the For heart specimens with DORV, a fibrous continuity was
posteroinferior quadrant of the VSD, and one with an found in 2 of 36 with DORV and subaortic VSD, 1 of 4 with
entirely posteroinferior muscular rim (Figure 4). DORV and doubly committed VSD, and zero with DORV
Fibrous continuity was found in 14 of 19 isolated outlet and subpulmonary VSD.
VSDs (74%), 47 of 71 specimens with TOF (66%), 19 of
49 specimens with TOF-PA (39%), 3 of 65 specimens Presence of an Outlet Septum
with DORV (4.6%), 1 of 54 specimens with CAT (1.8%), The outlet septum could be analyzed in all hearts except 2
and zero of the 16 specimens IAA with a VSD with TOF-PA because of previous surgery. A muscular
(P <.0001) (Figure 5). In all these specimens, the fibrous outlet septum could be demonstrated in 16 of 19 with outlet
continuity between the aortic (or truncal) and tricuspid VSD (84%), 68 of 71 with TOF (96%), 20 of 49 with
valves represented the posteroinferior border of the VSD: TOF-PA (40%), 61 of 65 with DORV (94%), zero with
The posteroinferior limb of the septal band was not fused CAT, and 13 of 16 with IAA (81%) (P <.0001).
with the ventriculo-infundibular fold. This fibrous continu- In TOF and TOF-PA, the outlet septum, when present,
ity always involved the anterior leaflet, and not the septal was always malaligned with the rest of the septum and
leaflet, of the tricuspid valve (Figure 4). deviated anteriorly. The inability to identify an outlet
In all of the morphologically normal hearts studied as septum in 60% of TOF-PA was probably due to its
controls, the posteroinferior limb of the septal band was excessive anterior deviation, making it indistinguishable
always separated from the ventriculo-infundibular fold by from the anterior wall of the right ventricle. The outlet

684 The Journal of Thoracic and Cardiovascular Surgery c March 2015

Mostefa-Kara et al Congenital Heart Disease: Fontan

CHD
FIGURE 3. Localization of the VSD in heart specimens with various outflow tract defects: TOF-PA (A), isolated outlet VSD (B), TOF (C), DORV with
subaortic VSD (D), DORV with subpulmonary VSD (E), CAT (F), and IAA type B viewed from the right ventricle (G). The localization of the VSD is always
the same, in the outlet of the right ventricle, between the 2 limbs of the septal band (anterosuperior and posteroinferior) (yellow Y). AO, Aorta; SB, septal
band; PV, pulmonary valve; TrV, truncal valve; TV, tricuspid valve.

septum was fibrous and not muscular in 2 hearts with TOF, It was deviated anteriorly, creating subpulmonary
and this fibrous remnant created valvar pulmonary obstruc- obstruction, in 31 of 36 specimens (Fallot-type DORV).
tion by restricting the diameter of the pulmonary annulus. In DORV with subpulmonary VSD, the outlet septum was
One heart with TOF and absent pulmonary valve had total always muscular and was deviated posteriorly, creating
absence of outlet septum with contiguous pulmonary and subaortic obstruction, in 10 of 25 specimens.
aortic valve without any fibrous remnant. In IAA type B, 3 hearts had a doubly committed VSD
In DORV with doubly committed VSD, the outlet septum with fibrous outlet septum and subaortic obstruction.
was always fibrous (fibrous raphe between the aortic and the Whether muscular or fibrous, the outlet septum was
pulmonary valve) and not muscular. In DORV with always malaligned with the rest of the ventricular septum
subaortic VSD, the outlet septum was always muscular and deviated posteriorly, creating a subaortic obstruction
and malaligned with the rest of the ventricular septum. (Figure E1).

FIGURE 4. Two heart specimens with TOF-PA. A, The outlet VSD, located between the 2 limbs of the septal band (yellow Y), has entirely muscular rims.
B, Outlet VSD (yellow Y) with fibrous continuity between the aortic and tricuspid valves. This continuity involves almost exclusively the anterior leaflet of
the tricuspid valve, disrupting the muscular continuity between the ventriculo-infundibular fold and the posteroinferior limb of the septal band. AO, Aorta;
ATL, anterior tricuspid leaflet; PTL, posterior tricuspid leaflet; SB, septal band; STL, septal tricuspid leaflet.

The Journal of Thoracic and Cardiovascular Surgery c Volume 149, Number 3 685
 Congenital Heart Disease: Fontan Mostefa-Kara et al

Although this type of VSD was common to all outflow

tract defects examined, we have found that its anatomic
variants are distributed differently according to the defect
involved, especially when focusing on the posteroinferior
rim and particularly on the aortic-tricuspid fibrous
continuity. We show that this continuity occurs with
different frequency among the various outflow tract defects
studied: The highest rate was found in isolated outlet VSD,
then decreasing progressively from TOF to TOF-PA, then
DORV, and to become exceedingly rare in CAT and absent
in IAA type B. These differences suggest an anatomic
CHD

FIGURE 5. The rate of fibrous continuity between the aortic and tricuspid continuum from isolated outlet VSD to IAA type B, rather
valves in the heart specimens with outflow tract defects. The highest rate than distinct physiologic phenotypes, related to various
was found in the group with isolated outlet VSD, then in TOF and degrees of abnormal rotation of the outflow tract: minimal
TOF-PA, rarely in DORV and CAT, and absent in IAA type B. in isolated outlet VSD; incomplete in TOF, TOF-PA, and
CAT, Common arterial trunk; DORV, double outlet right ventricle; DORV; absent in CAT; and probably excessive in IAA
IAA, interruption of the aortic arch; PA, pulmonary atresia; VSD, ventricu- type B. We recently demonstrated that the pattern of the
lar septal defect.
coronary orifices in outflow tract defects varies in a similar
way according to the degree of lack of rotation of the
DISCUSSION outflow tract during cardiac development.29 The anatomic
The VSD in outflow tract defects always opens in the variations in the posteroinferior rim of the VSD found in
outlet of the right ventricle, between the 2 limbs of the the group of cardiac defects in the current study confirm
septal band. The defects observed experimentally after these findings.
ablation of the cardiac neural crest include complete The high rate of aortic-tricuspid continuity found in
absence of septation of the outflow tract (CAT) and isolated outlet VSD is reminiscent of the anatomic
malalignment defects (DORV with subaortic, doubly characteristics of the normal heart. In the normal heart,
committed, or subpulmonary VSD; TOF and variants; the rotation of the outflow tract is complete, and the
overriding aorta; and IAA type B).3 wedging of the aorta between the tricuspid and mitral valves
CAT is defined by a single vessel originating from the allows not only for the mitro-aortic fibrous continuity but
heart, with a common arterial valve, producing the coronary also for the tricuspid-aortic continuity through the
arteries, at least 1 pulmonary artery, and systemic arteries.4 atrioventricular part of the membranous septum.9 This
Although rare cases with intact septum have been fibrous continuity can be seen from the right ventricular
described,21 CAT almost always includes an outlet VSD side below the ventriculo-infundibular fold, involving the
located between the 2 limbs of the septal band.22 There is anterior leaflet of the tricuspid valve, and disrupting the
no outlet septum; the superior rim of the VSD is formed muscular fusion between the ventriculo-infundibular
by the common arterial valve. fold and the posteroinferior limb of the septal band or
Malalignment defects are due to a lack of elongation of septomarginal trabeculation.20
the outflow tract during the different phases of cardiac The development of the ventricular septum has been
looping,3 preventing normal wedging of the aortic valve thoroughly analyzed in murine embryonic hearts at
between the tricuspid and mitral valves, with an outflow different stages of development, using high-resolution
tract that is shorter and straighter than normal.2 This episcopic microscopy.9 The results of this study are in
phenotype is due to a lack of addition of myocardial cells accordance with our anatomic findings. Ventricular
to the developing outflow tract by the secondary heart septation is achieved when the rotation of the aortic root
field.23,24 All malalignment defects include a VSD due and its transfer toward the left ventricle are complete. The
to the malalignment and the absence of fusion between perfect alignment between the outlet septum and the
the outlet septum and the rest of the ventricular septum, muscular septum then permits the fusion between the outlet
the outlet septum then being an exclusively right septum, formed by the outflow tract cushions, and the septal
ventricular structure.2,9 All authors agree that this band between its 2 limbs. The atrioventricular endocardial
VSD is cradled between the 2 limbs of the septal cushion tissue and the left outflow tract cushion will
band.17-19,22,25-27 Indeed, we found such an outlet VSD ultimately close the remaining small communication
in all the heart specimens studied. The only exception between the 2 ventricles, forming the membranous part of
was seen in 1 heart with IAA type B that had no VSD. the ventricular septum.9 In other words, it is the complete
Such cases, although rare, have been described in the wedging of the aorta that permits the formation of the
literature.28 membranous septum. We can infer from this study that

686 The Journal of Thoracic and Cardiovascular Surgery c March 2015

Mostefa-Kara et al Congenital Heart Disease: Fontan

the rotation or wedging may be less achieved in the To help solve this semantic problem, we analyzed 26
subgroup of outlet VSDs with entirely muscular borders hearts with isolated central perimembranous VSD present
than in outlet VSDs with tricuspid-to-aortic fibrous in our anatomic collection. All of these VSDs were located
continuity. This is consistent with our results, the rate of behind the septal leaflet of the tricuspid valve, under the
VSD with entirely muscular borders increasing from posteroinferior limb of the septal band, and not between
isolated outlet VSD to CAT and IAA type B. This reinforces the 2 limbs of the septal band. The outlet septum was intact
the hypothesis of an anatomic continuum according to the and completely fused with the septal band. In addition, we
degree of rotation of the outflow tract. observed that the fibrous continuity between the leaflets of
the tricuspid and the aortic valve was different in outlet
VSDs and in isolated ‘‘central’’ perimembranous VSDs:
Ventricular Septal Defect in Transposition of the
In the former, the anterior leaflet of the tricuspid valve
Great Arteries

CHD
was concerned, whereas in the latter, the fibrous continuity
To complete this study, we analyzed separately 104
involved almost exclusively the septal leaflet of the
hearts with TGA and associated VSD. In this group, the
tricuspid valve. This morphologic observation confirms
VSD was from the outlet type, cradled between the 2 limbs
the blatant anatomic difference between these 2 types of
of the septal band, in only 40% of the specimens (42/104).
the VSDs and underlines the risk of confusion created by
The posteroinferior rim of the VSD was always muscular, as
using the term ‘‘perimembranous’’ as the first determinant
in DORV with subpulmonary VSD. The outlet septum was
of the VSD. However, the presence of a fibrous continuity
muscular in 39 of these 42 specimens and fibrous in 3
at the posteroinferior rim of the VSD is important for the
specimens. Other VSDs were muscular in 26 specimens,
surgeon because it makes the conduction axis vulnerable
‘‘central’’ perimembranous in 27 specimens, and from the
during surgery and therefore must be described specifically
inlet type in 9 specimens. The fact that, contrary to other
in the preoperative assessment of the defect.
outflow tract defects, the VSD in TGA is not always from
the outlet type underlines the probable role of the laterality CONCLUSIONS
genes in the morphogenesis of TGA.12-14 This anatomic approach provides new perspectives on the
group of outflow tract defects, especially the subgroup of
Is the Ventricular Septal Defect in Outflow Tract cardiac neural crest defects. In all of the specimens studied,
Defects Perimembranous? the VSD always opened in the outlet of the right ventricle,
Although the inferior rim of the VSD in TOF is cradled between the 2 limbs of the septal band, irrespective
unequivocally described by all authors as formed by the of the presence or absence of a fibrous continuity between
superior part of the septal band and its 2 limbs, it is also the aortic and tricuspid valves, and the presence of an outlet
considered to be paramembranous17 or perimembranous18 septum. Although the VSD is always the outlet type, there
because of the presence of a fibrous aorto-tricuspid are some anatomic differences that seem to be related to
continuity in the majority of the cases. Outlet VSDs were the degree of rotation of the outflow tract during heart
classified by the European school as 3 types: (1) perimem- development. This anatomic approach places the outlet
branous, with fibrous continuity between the leaflets of VSD as a cornerstone of the outflow tract defects,
the aortic valve and those of an atrioventricular valve; anatomically different from the isolated central
(2) muscular, with entirely muscular borders; and perimembranous VSD. This may help us to better
(3) subarterial, with fibrous continuity between the leaflets understand the anatomy of the VSDs and to clarify their
of the aortic and pulmonary valves.30,31 To paraphrase classification and terminology.
these authors, one could say that these 3 types of outlet
VSDs are related in comparable fashion to the limbs of Conflict of Interest Statement
the septal band.30 To clarify terms and to differentiate this Authors have nothing to disclose with regard to commercial
type of VSD from the isolated, or ‘‘central,’’ perimembra- support.
nous VSD,20,32 we propose to rename the outlet VSD
with fibrous continuity between the aortic and tricuspid References
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CHD

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688 The Journal of Thoracic and Cardiovascular Surgery c March 2015

Mostefa-Kara et al Congenital Heart Disease: Fontan

CHD
FIGURE E1. Heart specimen with IAA type B viewed from the left
ventricle. The VSD is an outlet VSD. The outlet septum (*) is deviated pos-
teriorly, creating a subaortic obstruction. Ao, Aorta; MV, mitral valve; VSD,
ventricular septal defect.

The Journal of Thoracic and Cardiovascular Surgery c Volume 149, Number 3 688.e1