ECHO ROUNDS

Transesophageal Echocardiography Detection of

Undiagnosed Multiple Muscular Ventricular Septal
Defects with Alteration of Shunt Flow by Right
Ventricular Pacing After an Arterial Switch Operation
in a Neonate
Satoshi Kurokawa, MD, Miki Taneoka, MD, Hidekazu Imai, MD, Hiroshi Baba, MD,
and Minoru Nomura, MD

12-day-old female with transposition of the great

arteries and ventricular septal defect (VSD) underwent an arterial switch operation and VSD closure.
Written informed consent for this presentation was obtained from her parents. Transesophageal echocardiography (TEE) during the pre-cardiopulmonary bypass (CPB)
period confirmed cardiac pathologies consistent with
those diagnosed preoperatively, including a large perimembranous VSD and a mildly hypoplastic left branch
pulmonary artery (PA) that did not appear to require
angioplasty (2.5 m/s of peak velocity on transthoracic
echocardiography (TTE)). During the separation from
CPB, however, the TEE detected multiple VSDs with
left-to-right (L-R) shunting in the apical trabecular septum. These VSDs had not been detected by any of the
previous examinations (preoperative TTE, preoperative
cardiac catheterization, or pre-CPB TEE) or by direct
visual assessment during surgery. Because they were so
widely distributed over the trabecular muscular interventricular septum (IVS), it appeared that their surgical
closure would be very difficult to accomplish. Meanwhile, preexisting hypoplastic PA in conjunction with
distortion of the branch PAs after the Lecompte maneuver resulted in significant PA obstruction (left PA velocity of 3.2 m/s, right PA velocity of 3.0 m/s). At the same
time, the aortic root pressure was 62 mm Hg and the
right ventricular (RV) pressure was estimated at 58
mm Hg on the basis of a tricuspid regurgitation flow
velocity of 3.4 m/s. Thus, pulmonary overcirculation
was prevented by mechanisms similar to those seen after
PA banding. On the basis of these findings, we concluded that no additional surgery was indicated.
As the patient was being weaned from CPB, she suffered
from intermittent high-degree atrioventricular block. Our
initial attempt to treat the block by atrioventricular sequential

From the Faculty of Medicine, Department of Anesthesiology, Tokyo

Womens Medical University, Tokyo, Japan.
Accepted for publication March 18, 2011.
Funding: None.
The authors declare no conflicts of interest.
Supplemental digital content is available for this article. Direct URL citations
appear in the printed text and are provided in the HTML and PDF versions
of this article on the journals Web site (www.anesthesia-analgesia.org).
Reprints will not be available from the authors.
Address correspondence to Satoshi Kurokawa, MD, Tokyo Womens Medical University, Faculty of Medicine, Department of Anesthesiology, 8-1
Kawada-cho, Shinjuku-ku, Tokyo, Japan 162-8666. Address e-mail to
satokuro@sea.plala.or.jp.
Copyright 2011 International Anesthesia Research Society
DOI: 10.1213/ANE.0b013e31821e9765

August 2011 Volume 113 Number 2

pacing achieved only RV apical pacing, because the elevated

threshold prevented atrial capture. Later, after the patient was
smoothly weaned from CPB with sinus rhythm, her arterial
blood oxygen saturation suddenly deteriorated and her circulation collapsed. CPB was quickly reinstituted to stabilize
circulation. Thereafter, the same event recurred during
each of several attempts to wean the patient from CPB.
Eventually, TEE confirmed a switch of the direction of the
shunt from L-R in sinus rhythm to mainly right-to-left (R-L)
in RV pacing (Figure 1) (Video 1, see Supplemental Digital
Content 1, http://links.lww.com/AA/A282; see Appendix
for video caption). We then infused isoproterenol to restore
a persistent sinus rhythm. Once a sinus rhythm was
achieved, no R-L shunt developed, and her circulation
remained stable.
New information on cardiac pathology provided from
intraoperative TEE has the potential to alter surgical management in approximately 2%7% of patients with congenital heart disease.1,2 In another 2%, visual assessment during
surgery reveals new abnormalities.2 The detection of undiagnosed lesions after separation from CPB may only be
possible in rare instances. In our patient, the multiple
muscular VSDs were detected only after the surgical procedures were completed. The increased differential between the RV pressure and left ventricular (LV) pressure
before the termination of the CPB probably increased the
sensitivity of the color Doppler in VSD detection. Stauder et
al. reported a patient with perimembranous VSD in whom
several TTEs and repeated cardiac catheterizations failed to
detect an additional large apical trabecular VSD (magnetic
resonance imaging incidentally discovered the VSD).3 We
must be aware that apical muscular VSD is a lesion very
likely to be missed in preoperative examinations, especially
in cases with high RV pressure equivalent to the LV
pressure.
TEE also detected the development of bilateral PA
branch stenoses, a condition that prevented pulmonary
overcirculation. This finding contributed to our conclusion that no additional surgery was indicated. In the
Lecompte maneuver, PA bifurcation is anteriorly translocated to the neo-aorta.4 The maneuver has been reported to cause the branch PA stenosis near the point of
either left or right PA origin, or at both points, via the
distortion of the branch PAs caused by the anterior
position of the bifurcation.5
Our case exhibited a marked R-L shunt during early to
mid systole and a marked L-R shunt during late systole in
RV pacing, but a somewhat less marked L-R shunt during late
systole in sinus rhythm. RV apical pacing is a well-established
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ECHO ROUNDS

Figure 1. Midesophageal 4-chamber view demonstrating the presence of multiple ventricular septal defects (VSDs) and cardiac rhythm
dependent changes in both the direction and degree of shunt flow. In right ventricular (RV) apical pacing, a remarkable right-to-left shunt through
multiple VSDs (white arrows) was seen during early to mid systole (left upper panel), and a marked left-to-right shunt was observed during late
systole (right upper panel). Contrary to RV pacing, a negligibly low shunt was present during early to mid systole (left lower panel), and a less
marked left-to-right shunt was seen during late systole in sinus rhythm (right lower panel).

cause of 2 types of cardiac mechanical dyssynchronization:

dyssynchrony between LV and RV contractions (interventricular dyssynchrony) and dyssynchronized contraction
within the LV (intraventricular dyssynchrony).6 Prinzen et
al. demonstrated an uneven distribution of systolic myofiber
strain within the LV not only in time, but also in strength,
during ventricular pacing.7 In their study, a very small
biphasic strain with peaks in the early and late ejection
phase was obtained in the IVS, and a large strain with a
peak in the late ejection phase was obtained in the LV
lateral wall in RV apical pacing. On this basis, we
speculate that 2 possible mechanisms may have accounted for the changes in both the direction and severity of the shunt through the VSD in our patient. First,
interventricular dyssynchrony might have induced an
early increase of RV pressure before the increase of the
LV pressure, resulting in R-L shunt during early to mid
systole. Second, intraventricular dyssynchrony accompanied by poor contraction of the IVS may have prevented
the VSDs from contracting to minimum size during both
RV contraction in early systole and LV lateral wall
contraction in late systole. We should recognize that RV
apical pacing can cause changes in both the direction and
degree of the shunt through the VSD.
In summary, intraoperative TEE is a valuable technique
for detecting undiagnosed VSDs or residual defects, for
identifying unexpected changes in the direction and degree
of shunt flow during ventricular pacing, and for assisting in
surgical decision making.

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APPENDIX: VIDEO CAPTION

Video 1. Midesophageal 4-chamber view demonstrating ventricular
septal defect (VSD) shunt flow in right ventricular (RV) pacing and in
sinus rhythm. A remarkable VSD shunt from the RV to left ventricle
(LV) was observed during early to mid systole, and a marked shunt in
the opposite direction, from LV to RV, was observed during late
systole in RV pacing. On the other hand, a less-marked left-to-right
shunt was seen during late systole in sinus rhythm.
DISCLOSURES

Name: Satoshi Kurokawa, MD.

Contribution: This author helped conduct the study, analyze
the data, write the manuscript, and conduct the anesthetic
management, transesophageal echocardiography, and manuscript preparation.
Attestation: Satoshi Kurokawa approved the final manuscript.
Name: Miki Taneoka, MD.
Contribution: This author helped conduct the study and
conduct the anesthetic management.
Attestation: Miki Taneoka approved the final manuscript.
Name: Hidekazu Imai, MD.
Contribution: This author helped conduct the study and
conduct the transesophageal echocardiography.
Attestation: Hidekazu Imai approved the final manuscript.
Name: Hiroshi Baba, MD.
Contribution: This author helped conduct the study and
conduct the anesthetic management.
Attestation: Hiroshi Baba approved the final manuscript.
Name: Minoru Nomura, MD.
Contribution: This author helped write the manuscript.
Attestation: Minoru Nomura approved the final manuscript.

ANESTHESIA & ANALGESIA

Detection of Undiagnosed Multiple Muscular VSDs

REFERENCES
1. Bettex DA, Schmidlin D, Bernath MA, Pretre R, Hurni M, Jenni
R, Chassot PG, Schmid ER. Intraoperative transesophageal
echocardiography in pediatric congenital cardiac surgery: a
two-center observational study. Anesth Analg 2003;97:1275 82
2. Sheil MLK, Baines DB. Intraoperative transesophageal echocardiography for paediatric cardiac surgeryan audit of 200 cases.
Anaesth Intensive Care 1999;27:5915
3. Stauder NI, Miller S, Scheule AM, Ziemer G, Claussen CD. MRI
diagnosis of a previously undiagnosed large trabecular ventricular septal defect in an adult after multiple catheterizations and
angiocardiograms. Br J Radiol 2001;74:280 2
4. Lecompte Y, Zannini L, Hazan E, Jarreau MM, Bex JP, Viet Tu T,
Neveux Y. Anatomic correction of transposition of the great
arteries. New technique without use of a prosthetic conduit.
J Thorac Cardiovasc Surg 1981;82:629 31

Clinicians Key Teaching Points

5. Beek FJA, Beekman RP, Dillon EH, Mali WPTM, Meiners LC,
Kramer PPG, Meyboom EJ. MRI of the pulmonary artery after
arterial switch operation for transposition of the great arteries.
Pediatr Radiol 1993;23:335 40
6. Tops LF, Schalji MJ, Bax JJ. The effects of right ventricular apical
pacing on ventricular function and dyssynchrony. Implication
for therapy. J Am Coll Cardiol 2009;54:764 76
7. Prinzen FW, Hunter WC, Wyman BT, McVeigh ER. Mapping of
regional myocardial strain and work during ventricular pacing:
experimental study using magnetic resonance imaging tagging.
J Am Coll Cardiol 1999;33:1735 42

By Nikolaos J. Skubas, MD, Roman M. Sniecinski, MD,

and Martin J. London, MD

Transposition of the great arteries (TGA) can be surgically corrected with the arterial switch operation. The great
vessels are anastomosed to the correct anatomic ventricle by bringing the main pulmonary artery (PA) and its branches
anteriorly to connect to the right ventricle (RV) via the aortic root, while the distal aorta is connected to the left ventricle
(LV) via the PA root, to which the coronary arteries are also reattached. The congenital communication (atrial or
ventricular septal defect [VSD]), which allowed mixing between pulmonary and systemic circulation, is also closed.
Muscular VSDs located in the apical part of the interventricular septum (IVS) may be undetected until after separation
from cardiopulmonary bypass (CPB). This is because rhythm or pressure changes can alter the pressure gradient
across the IVS and facilitate abnormal flow between the RV and LV. Color flow Doppler placed across the IVS in the
midesophageal views is important to demonstrate the presence of such communications.
In this case, after surgical repair in a neonate with TGA and VSD with left-to-right shunt, periods of RV epicardial pacing
caused contraction delays between the RV and LV (interventricular dyssynchrony) and the IVS and lateral LV walls
(intraventricular dyssynchrony). This increased the RV systolic pressure, caused right-to-left shunt via the undetected
apical VSDs, hypoxemia and hemodynamic collapse.
Hemodynamic conditions can become significantly altered after cardiac surgery and CPB. As this case illustrates, an
intraoperative transesophageal echocardiography examination is instrumental for detecting pathology, such as
intracardiac shunts, that may not have been obvious, or even existed, in the patients preoperative state.

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