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日本小児循環器学会雑誌 1巻2号214～219頁（1986年）
Echocardiographic Features of So－called Aneurysm of Membranous
Septum in d－Transposition of the Great Arteries
Ganesja M． Harimurti， Gengi Satomi， Kazuhiro Mori， Kan Touyama，
Takayuki Konishi， Hirofumi Tomimatsu， Michiko Ando
and Atsuyoshi Takao
Department of Pediatric Cardiology， Heart lnstitute of Japan， Tokyo Women’s Medical College
Department of Cardiology， Dr． Cipto Mangunkusumo Hospital， University of lndonesia
Summary
The so－called aneurysm of membraneous ventri－
cular septum（AMS）in d－TGA may cause LV out一
且ow tract obstruction， which presents as a
technical procedural problem in determining the
type of surgery．
In 5 patients with d－TGA， AMS was recognized
by two－dimensional echocardiography before
catheterization and angiographic studies． Apical
four－chamber and parasternal long axis views
appeared to be very sensitive in visualizing them．
The appearances of the AMS in the cardiac cycle
varied；in l case it apPeared only during systolic
phase， but in the other 4 cases during systolic and
diastolic phase． These findings were compared
with the pressure gradient pattern between RV and
LV during each cardiac cycle． This indicated that
the appearances of the AMS were related to the
pressure gradient pattern between RV and LV in
each cardiac cycle．
Introduction
So－called aneurysm of membranous ventricular
septum（AMS）in d－Transposition of the great
arteries（d－TGA）may cause left ventricular outflow
tract obstruction， and its presence apPears as one
of the determining factors in deciding the type of
surgery1． The findings of left ventricular outflow
tract obstruction in d－TGA with M－mode echo－
cardiography has been published by several inves－
tigators2 一 4， but 2－dimensional echocardiography
could detect this more accurately． Snider et al5 and
別刷請求先 （〒162）東京都新宿区河田町10
東京女子医科大学心研小児科
里見 元義
Gussenhoven et al6 described two－dimensional
echocardiographic pattern of aneurysms of the
interventricular septum， and detection of left ven－
tricular outflow tract obstruction by two－dimen－
sional echocardiography have also been
discussed7，s．
In this paper we present M－mode and two－dimen－
sional echocardiographic findings of five cases with
AMS in d－TGA， compared with catheterization
data and cineangiograms， and to discuss the best
technique in visualizing them．
Materials and Methods
From June 1984 to July 1985，65 cases with d－
TGA have been evaluated echocariographically at
The Heart Institute of Japan， Tokyo Women’s
Medical College． In five cases（7％）with d－TGA，
AMS were visualized by two－dimensional echo－
cardiography． Their ages ranged from 3 to ll
months；1female and 4 males． The diagnosis of d－
TGA were confirmed echocardiographically before
catheterization and angiographic study in all cases．
Two－dimensional echocardiography was per－
formed using a Toshiba Sonolayergraph model
SSH－11A， or Aloka Mechanical Sector Scanner
SSD－720 with a 5 MHz transducer． Two－dimen－
sional echocardiographic images were recorded on
a Sony Video tape recorder to be analyzed later． M－
mode echocardiograms were recorded at 25mm／s
and 50mm／s using a strip chart recorder．
Every infant underwent a complete two－dimen－
sional echocardiographic examination based on
Satomi’s systematic two－dimensional echocardio－
graphic apProach for diagnosis of congenital heart
disease9． All cases had situs solitus， concordant
atrio－ventricular connection and discordant ven一
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日小循誌 1（2），1986
triculo－arterial connection． In cases 2 and 4， the
examination was carried out after palliative
surgery． Cases 2，4， and 5 have been surgically cor－
rected using Jatene’s procedure （Lecompte’s
modification）10・11．
Result
Ca thete riza tion and angiogrmph ic廊飽8s
Table l illustrates the catheterization data． Case
lhad a pressure gradient between the main pul－
monary artery（MPA）and left ventricle（LV）about
20mmHg， and in case 2 it was 13mmHg． The
other 3 cases did not show any pressure gradient
between MPA and LV． The pressure difference be－
tween right ventricle（RV）and LV were variable，
but the RV pressure was higher than the LV pres・
sure in all the cases． In cases l and 2， no ventricular
septal defect（VSD）was detected angiographically
（Table 2）， and only in case 2， AMS was visualized．
Figure l was a RV angiography in lateral projec－
tion from case 2， showing the presence of the AMS
protruding into LV couflow tract during systole．
Surgicalfinding
Three out of five cases were operated． The pro－
trusion of membranous septum without defect was
detected in one of them（case 2）during surgery． In
Table 1． Catheterization findings of 5 cases
with AMS in d・TGA．
systolic pre舗ロe（㎜Hg）Case
RVp LVp PG（LVp－PAp）
1． 4M M
2． 9M F
3． 3M M
4．11M M5． 6M M
85
93
80
82
77
51
75
72
80
67
20
13
000
一
PAB B－H
－
PAB
一
PAB＝pulmonary artery banding
B－H＝Blalock－Hanlon op．
Table 2． Angiographic findings．
VSD Protrusion
Case 1 一 2
一 十
3 十 一
4 十一
5 十 一
215－（79）
another two cases（cases 4 and 5）， the fibrous
tissue－tags were recognized at around the ventri－
cular septal defects．
〃二〃204θ励oα〃吻9吻吻〔ゾ・4ルfS
In M－mode echocardiography， AMS was seen as
multiple layers and coarse且uttering echos viewed
between the anterior mitral leaflet and the root of
pulmonary artery． Figure 2 shows the coarse flut－
tering echos of the AMS． Figure 3 is an illustration
RV grophy （ iateral）
T．1．9♪∧
Fig．1 Lateral RV graphy of case 2 showing the protrusion
of AMS into the LV outfiow tract．
lllillllltlllllHIIIIIIIIIIIIIUIIIMIIIIIIIIIilllllllllllBllllllll「川
一．一一〔〉，→・ら】～〔・“， ～‘’『’v’‥ ’〔♪’∀『wtl’』・で ’t’4醗鷺’鞠紺瞭・辞 違癒
rtLrWLrWN’tWWVvwv Y．1．3M
Fig．2M－mode echocardiographic picture showing coarse
且uttering of AMS．
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216－（80）
ll，i，’、／，，i，，！，，1川協川1，1，ll，、1繍，ll晶川1，細1，，，1‖，，il，，i川1、；，1，‖，，』ll，i，1，，1山，／・、，i、
H．E．6M
Fig．3 Two－dimensional and m－mode echocardiographic
pictures of the AMS（between arrows）．
of the M－mode echocardiographic pattern seen in
AMS． The movement of the AMS posteriorly into
the LV out且ow tract is shown in this figure．
TWO－di〃zensional echoca rdiograPhy〔ofAルfS
Parasterna l Short axis view
In parasternal short axis view， the AMS was
visualized protruding into LV in only l case（case
5），Fig．4A．
Apicalfour一吻〃2ber・view
Slight upward and anterior angulation from the
apical four－chamber view colud visualize the mem－
branous part of the interventricular septum． With
this view， the AMS was seen as a localised protru－
sion of the membranous ventricular septum into
LV out且ow tract． This view could visualize AMS in
all the cases． Figure 4B illustrates the two－dimen－
sional echocardiographic features of AMS using
the apical four－chamber view．
Parasterna〃oκ9磁s舵％
In parasternal long axis view， the AMS was seen
日本小児循環器学会雑誌 第1巻 第2号
short oxis view
four chamber view
long axis view
Fig．4AMS in several two－dimensional views．
Table 3． Two－dimensional echocardiographic
windows visualizing the AMS in 5 cases．
Echocardiographic windows
Case
long axis ・
Vlew Vlew Vlew
1 十 十
2一
十 十
3一
十 十
4一 十 十
5 十 十 十
as a localized protrusion of the membranous ven－
tricular septum into LV outfiow tract． With this
view， AMS was also visualized in all the cases．
Figure 4C is an illustration of the protrusion of the
membranous septum beneath the pulmonary valve．
AMS was visualized both in the apical four一
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昭和61年3月1日
CASE 1
2
’34
5
Fig．5Timing of appearances of thor AMS in each cardiac
cycle．
態編嚢藩三難影
口∫＼
晶，㌔，1，、li，1，1・，1，日川1，，1、，、、lll，品、，1h，1晶、，1、i、k，ul，ll，ll。1，ii：k・iilwi｛川／山・ili・IIU・／・
H．E．6M
Fig．6M・mode echocardiography of case 5 showing that
the abnormal echo moved posteriorly just before the peak
of T wave， and disappeared at the end of P wave．
chamber and parasternal long axis views in all the
cases（Table 3）． Only l case showed AMS in para－
sternal short axis view．
Timing ofaPPearance ofMAS
Figure 5 shows roughly the timing of appear－
ances of the AMS in each cardiac cycle in all the
cases． It was apparent that differences in the
timing of appearances of AMS were present． In
one case， the AMS appeared only during systolic
phase， but in the other 4 cases it protruded into LV
outflow tract during systole and diastole． This find－
ing was obtained by analysing the two－dimensional
echocardiograms， frame by frame． Figure 6 shows
an echocardiogram of case 5． The abnormal echo
217－（81）
was seen to be moved posteiorly just before the
peak of T wave， and disappeared at the end of P
wave． Thus it is interpreted that the AMS pro－
truded into LV out且ow tract during systole and
diastole；this matched well with the timing of ap－
pearance of AMS in two－dimensional echo－
cardiography．
Discussion
Aneurysm of the membranous part of the inter－
ventricular septum is a small conical projection of
thin membrane which arises from the margin of
VSD， and protrudes in systole i2． In the heart where
no discordance of ventriculoarterial connection is
present， and the RV functions as a venous
chamber， the presence of AMS in the ventricular
outflow tract does not cause significant obstruc－
tion， except in the situation where the protrusion is
very large， But in d－TGA， the higher pressure of
the systemic RV could push such protrusion， and
therefore may produce obstruction． Only 20f our
cases（40（70）showed pressure gradient between
MPA and LV， compared with 60f 8 cases（75（70）in
the finding of Vidne et alis． In cases l and 2， VSD
was not visualized by angiography；but in case 2 it
was detected during surgery． It may be that the
AMS occluded the VSD， resulting functional
closure． Several investigators have reported about
the formation of membranous septal aneurysm
resulting in diminution and spontaneous closure of
VSDi4・i5．
AMS was seen by angiography in only l case．
Sansa et a116 found significant LV outflow tract
obstruction in 33（70 using cineangiograms of 225
TGA children with or without coexisting VSD．
Echocardiography could detect AMS accurately，
and two－dimensional echocardiography is a sen－
sitive method to visualize such structure． In para－
sternal short axis view， AMS was visualized in only
lcase， but apical four－chamber and parasternal
long axis views could visualize them in all cases．
Thus， in conclusion， apical four－chamber and para－
sternal long axis views appeared as the best way to
visualize AMS， Slight upward and anterior angula－
tion was the ideal technuque to observe the aneury一
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218－（82）
80
40
）（
pressure
Pressure
Fig．7 RV and LV pressure tracings of case 5． RV pressure
was higher than LV pressure during systole and diastole．
smal protrusion of the membranous part of the
interventricular septum． As for the timing of the
appearance of AMS， it is speculated that this
phenomenon could be in accordance with the
differences of the pressure gradient between RV
and LV during each cardiac cycle． To prove this，
we traced the pressure tracings of both RV and LV
of the cases and superimposed them． It revealed
that the RV pressure was higher than the LV pres－
sure during systole in case 2， and during systole
and diastole in the other 4 cases． Fig 7 shows the
superimposed LV and RV pressure tracings of case
5showing that the RV pressure is higher than the
LV pressure during systole and diastole． So， the
findings indicated that the appearances of AMS in
each cardiac cycle was related to the pressure
difference pattern between RV and LV．
References
1．Stark J：Concordant transposition－other operations．
In Surgery of Congenital Heart Defects．（Stark J and
De Leval M， ED．）Grune＆Straton， London， New
York，1983， p．375．
2．Nanda NC， Gramiak R， Manning JA， Lipchik EO：
Echocardiographic features of subpulmonic obstnlc－
tion in dextro－transposition of the great vessels．
Circulation 51：515－521，1975
3．Aziz KU， Paul MH， Muster AJ：Echocardiographic
assessment of left ventricular out且ow tract in d－trans－
position of the great arteries． Am J Cardiol 41：543－
551，1978
4．Seward JB， Tajik AJ， Giuliani ER， Mair DD：
Aneurysm of the membranous ventricular septum in
transposition of the great arteries：Echo features
日本小児循環器学会雑誌 第1巻 第2号
5
6．
7
8
9
10．
11．
12．
13．
14．
15．
16．
（letter）． Circulation 54：161－2，1976
Snider AR， Silverman NH， Schiller NB， Ports TA：
Echocardiographic evaluation of ventricular septal
aneurysms． Circulation 59：920－926，1979
Gussenhoven WJ， DeRiele JAM， Scherpenzeel W，
Roelandt J：Echocardiographic pattern in aneurysm
of the membranous interventricular septum． Chest
77：541－543，1980
Williams RG， Fellows KE， Castaneda AR：Anatomic
types of subpulmonary stenosis in d－transposition of
the great arteries by echo and angiography（abstract）．
Am J cardiol 41：418，1978
Bierman FZ， Williams RG：Prospective diagnosis of
d－transposition of the great arteries in neonates by
subxyphoid， two－dimensional echocardiography．
Circulation 60：1496－1502，1979
Satomi G， Iwasa M， Minami Y， Takao A， Nakamura
K：Systemic two－dimensional echocardiographic
apProach for diagnosis of congenital heart disease． J
Cardiography 10：987－1001，1980（in Japanese with
English summary）．
Jatene AD， Fontes VF， Souza LCB， Paulista PP，
Abdulmassih Neto C， Sousa JEMR：Anatomic correc－
tion of transposition of the great arteries． J Thorac
Cardiovasc Surg 83；20－26，1982
Lecompte Y， Zannini L， Jerreau MM， Bex Jp， Viet
Tu T， Neveus JYI Anatomic correction of trnasposi－
tion of the great arteries． New technique without use
of prosthetic conduit． J Thorac Cardiovasc Surg 82：
629－631，1981
Rudolph AM：Congenital Diseases of the heart．
Chicago， year Book medical Publishers， Inc．1974， p
218．
Vidne BA， Subramanian S， Wagner HR：Aneurysm
of the membranous ventricular septum in transposi－
tion of the great arteroes． Circulation 53：157－161，
1976
Miora KP， Hildner FJ， Cohen LS， Narula OS， Samet
P：Aneurysm of the membranous ventricular septum：
amechanism for spontaneous closure of the ven－
tricular septal defect． N Engl J Med 283：58，1970
Nugent EW， Freedom RM， Rowe RD， Wagner HR，
Rees JK：Aneurysm of the membranous septum in
ventricle septal defect． Circulation 56（suppl I）：1－82，
1977
Sansa M，Tonkin IL， Bargeron LM Jr， Elliot LP：Left
ventricular out且ow tract obstruction in transposition
of the great arteries：An angiographic study of 74
cases． Am J Cardio144：88－95，1979
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昭和61年3月1日 219－（83）
大血管転i換症におけるいわゆる膜様部中隔瘤のエコー所見
（昭和60年7月27日受付）
（昭和60年12月3日受理）
東京女子医科大学附属日本心臓血圧研究所小児科
Ganesja M． Harimurti 里見 元義 森 一博 遠山 歓
小西 貴幸 富松 宏文 安藤美智子 高尾 篤良
key words：大血管転換症，膜様部中隔瘤，心エコー図
大血管転換症におけるいわゆる膜様部中隔瘤
（AMS）は，左室流出路の狭窄をきたすことがしられて
おり，時には手術手枝の決定に重要な情報を与える．
我々は，1984年7月から1985年6月までに65例の大
血管転換症に心エコー図検査を施行し，うち5例
（7％）にいわゆる膜様部中隔瘤を認めた．5例の内訳
は男4例，女1例で年齢は，3ヵ月から11ヵ月までで
あった．症例2と症例4は，肺動脈絞拒術とBlalock－
Taussig短絡術後に心エコー図検査を施行し，また症
例2，4および5に対しては後にJatene手術が行なわ
れた．カテーテル検査所見では，症例1では，左室と
肺動脈間に20mmHgの，また症例2では，13mmHgの
圧較差を認めた．他の3例では，左室と肺動脈間に圧
較差は認められなかった．右室と左室間の圧較差は一・
様ではなかったが全例において右室圧の方が左室圧よ
りも高かった．症例1と2では，造影検査上心室中隔
欠損は証明されず，また造影上いわゆる膜様部中隔瘤
が認められたのは症例2のみであった（Fig．1）．手術
を行なった3例のうち1例では心室間短絡はなかった
ものの膜様部中隔の瘤状の突出が，また他の2例では
心室中隔の周囲にTissue－tagの付着が手術時直視下
に確認された．
心エコー図所見
1．Mモード心エコー図所見：AMSは僧帽弁前尖
と肺動脈への左室流出路との間に多重の粗いflutter・
別刷請求先 （〒162）東京都新宿区市ケ谷河田町10
東京女子医科大学心研小児科 里見 元義
ingを有するエコーとして観察された（Figs．2and 3）．
AMSのエコーは，心室中隔よりも後方への動きとし
て認められた．
2．断層心エコー図所見：断層心エコー図上AMS
は，傍胸骨部からの短軸断面で1例に，心尖部四腔断
面を軽度頭側に起こした断面で5例全例に，傍胸骨部
からの左室長軸断面で5例全例に検出可能であった（Table 3）．
3．MSA出現のタイミング：MSA出現のタイミン
グは，症例によって異なっていた（Fig．5）．1例では
収縮期のみに出現したが，他の4例では，収縮期から
拡張期を通じて出現した．
今回の我々のシリーズでは，左室，肺動脈間に圧較
差を生じたものは，5例中2例（40％）とVidneらの
75％に比して少なかったが，完全大血管転換症におけ
るAMSは，手術方針決定の上で術前に正確に認識さ
れなけれぽならないものである．心エコー図上AMS
の認められた5症例のうちシネアンジナグラフィーで
も検出されたのは1例のみでAMSの診断のsen・
sitivityでは心エコー図検査の方が優れていると判断
される．Mモード心エコー図では肺動脈下の左室流出
路に心周期の様々のタイミソグで出現する粗い
flutteringを伴う多重エコーとして，また断層心エコー
図では，やや頭側に向けた心尖部四腔断面や傍胸骨部
からの長軸断面で肺動脈下の左室流出路に，瘤状に突
出する構造物として容易に認識することが可能であ
る．
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