European Journal of Cardio-Thoracic Surgery 50 (2016) 1001–1005
doi:10.1093/ejcts/ezw333 Advance Access publication 15 October 2016
Cite this article as: Nikaidoh H. Nikaidoh procedure: a perspective. Eur J Cardiothorac Surg 2016;50:1001–1005.
Nikaidoh procedure: a perspective
Hisashi Nikaidoh*
Department of Cardio-Thoracic Surgery, Cook Children’s Hospital, Fort Worth, TX, USA
* Corresponding author. Department of Cardio-Thoracic Surgery, Cook Children’s Hospital, 1500 Cooper Street, Floor 3, Fort Worth, TX 76104-2796, USA.
Tel: +1-682-8556400; fax: +1-682-8556101; e-mail: hnikaidoh@aol.com (H. Nikaidoh).
Keywords: Aortic translocation • Nikaidoh procedure • Complex transposition
BACKGROUND
In 1930, Katsuji Kato published a pathologic study on congenital
transposition of cardiac vessels, based on then the most exhaust-
ive collection of 86 previously reported ‘complete’ transpositions.
He also included his own observations on five cases at Children’s
Memorial Hospital (now the Ann and Robert H. Lurie Children’s
Hospital) in Chicago, IL, USA. He concluded that there was no ef-
fective treatment for these children [1]. At this same hospital in
1955, Thomas Baffes performed his first successful half venous
switch operation (Baffes procedure) on a 4-year-old girl with
transposition, without using cardiopulmonary bypass [2]. Several
years later, at the same institution, Farouk Idriss attempted two
arterial switch operations (ASOs) for transposition, but was un-
successful [3]. I had the unique privilege of working with Drs.
Baffes and Idriss while they were getting accustomed to the
newly described Mustard procedure for transposition [4].
Together, we were then challenged with increasingly complex
transpositions of the great arteries (TGA), which included ven-
tricular septal defect (VSD), and left ventricular outflow tract ob-
struction (LVOTO) or pulmonary stenosis (PS) [5, 6].
The advent of the ASO of Adib Jatene brought a new era in
surgical treatment of d-TGA in 1975 [7]. It took nearly a decade
for us to learn the appropriate application of this operation to
various forms of d-TGA. The only anatomic combination which
defied ASO was d-TGA with VSD and LVOTO, though this par-
ticular combination has been managed by the ingenious oper-
ation designed by Giancarlo Rastelli [8] in 1968 and reported the
next year.
BIRTH OF NIKAIDOH PROCEDURE
In the earlier part of the 1980s, I observed the tragic death of a
child who developed biventricular outflow obstruction as the late
complication of a Rastelli operation. I then recognized the short-
comings of the Rastelli operation, namely the vulnerable left ven-
tricle (LV) to aortic valve (AV) connection and the dependence
on a non-growing right ventricle (RV) to pulmonary artery (PA)
conduit which was impinged between the sternum and the heart.
These apparent shortcomings were verified by Kreutzer [9] and
others from Boston Children’s Hospital in 2000.
C The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
V
EDITORIAL
EDITORIAL
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In 1983, two of my patients with d-TGA with VSD and LVOTO
underwent aortic translocation (ATL) and biventricular outflow
tract reconstruction. In both patients, the aortic root was mobi-
lized circumferentially off the RV and was implanted to the LV
outflow area after the division of the stenotic pulmonary annulus
and conal septum. A large autogenous pericardial baffle con-
nected RV to PA. When this clinical success was reported in
1984, well-deserved credit was given to the previously described
concepts which contributed to the development of this operation
[10]. Neither of these original two patients experienced further
surgical intervention during the following 25 years.
The mid-term result of our experience was reported in 2007
by Yea et al. In this report, we recognized one early death among
19 patients, and no late death within the follow-up period of 30
years. None of our patients had LV outflow reintervention. The
RV outflow tract received reintervention in five patients, and
freedom from reintervention at 15 years was 64% [11].
NEW MODALITIES
As we became more familiar with coronary artery transfer
through increasing experience in ASO, ATL was performed more
routinely utilizing coronary artery transfer and the Lecompte
maneuver, as Morell et al. reported on their series of 12 patients
in 2005 [12].
Two years earlier, Yamagishi et al. reported a successful recon-
struction of biventricular outflow tracts by truncal mobilization
and 180-degree rotation in a 1-year-old patient with d-TGA,
VSD, and PS [13].
In 2007, Hu et al. [14] reported similar modification with cor-
onary artery transfer and the Lecompte maneuver and partial sal-
vage of pulmonary valve on four patients. This modification was
later renamed by Hu et al. [15] as double root translocation.
EXPANDED APPLICATIONS
In the original report in 1984, I envisioned this operation applying
only to d-TGA, VSD, and PS. The side-by-side spacial relationship
of the great arteries, such as in double outlet right ventricle
(DORV), seemed to me a contraindication. However, the surgeons’
1002 H. Nikaidoh / European Journal of Cardio-Thoracic Surgery

ingenuity and improved skills have expanded the application far

beyond my original expectations.
Hu et al. reported successful application of double root trans-
location in 12 patients with DORV, VSD and PS utilizing their
modification in 2010 [15].
Morell et al.’s original report in 2005 contained three corrected
TGA (ccTGA) patients, and even straddling atrioventricular valves
in 3 patients among their 12 patients who underwent ATL [12].
The more recent series of Kramer et al. [16] had 4 DORV and 2
ccTGA patients among the total of 21 of ATL.

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In 2008, Hraska [17] reported a successful application of ATL in
a 2-year-old girl with ccTGA, VSD, PS, and straddling of the tri-
cuspid valve in {I, D, D} anatomy.
Just this year, a successful application of ATL was reported in a
9-year-old boy with d-TGA, PS, complete atrioventricular septal
defect, and heterotaxy, by Sugiura et al. [18].
Also reported this year was a successful ATL in a 16-month-old
boy with TGA, VSD, and PS in a {S, D, L} anatomy, by Reeves et al.
[19].
Various coronary artery distributions, especially when a large
branch is coursing over the RV outflow tract, have been considered
contraindications for ATL. However, this has been overcome in a
report by Ugurlucan et al. [20]. In 2013, the author assisted Steven
R. Leonard in Denver, CO, USA, successfully performing ATL in an
8-month-old boy in whom the distal anterior descending coronary
artery was originating from the right coronary artery.

Figure 1: External morphologic features: dotted lines indicate incisions. Ao:

IDEALIZED NIKAIDOH PROCEDURE ascending aorta; LV: left ventricle; PA: main pulmonary artery; RA: right atrium;
RV: right ventricle; SVC: superior vena cava.

As much as the scope of ATL operations has expanded, there

should still be an ‘ideal’ candidate and technical principles for
this procedure. The author will attempt to describe such a pa-
tient. This scenario is not a fantasy, but is based on my personal
experience from several recent cases.
The ideal patient is an infant of several months of age with d-TGA,
VSD, PS, {S, D, D}, with normal venous anatomy, and preferably with-
out any palliative procedural history other than balloon atrial septos-
tomy. The undisturbed pericardial space gives the surgeon a better
view of important structures and the potential option of using au-
togenous pericardium for the repair. The great arteries are in an an-
teroposterior relationship and the coronary arteries don’t present any
technical challenges for posterior movement of the aortic root. The
preferred LVOTO is a small pulmonary annulus which will demand
only limited posterior movement for the aortic root, thus less likeli-
hood of coronary artery kinking or distortion. The ideal VSD is sub-
pulmonic. Also ideal will be bilateral normal atrioventricular valves.
The heart will be approached via midline sternotomy. The aorta
will be cannulated high in the ascending aorta, and bicaval cannu-
lation will start cardiopulmonary bypass. During the induction of
moderate hypothermia, the following necessary dissection should
be done in order to shorten the myocardial ischemic time, as the
procedure demands a long aortic cross clamp time. The ascending
aorta is fully mobilized and separated from the main PA down to
the base of the heart. The left and right coronary arteries are
encircled with vessel loops and mobilized at least several milli-
meters from their origin. Bilateral pulmonary arteries are fully
mobilized to the hilum in preparation for the Lecompte maneuver.
The arterial duct structure will be divided during this process.
Once the aorta is cross clamped and antegrade cardioplegia is
induced, the aortic root will be mobilized including a free
anterior RV wall which will be used as a VSD patch. During this
mobilization, special attention has to be paid not to injure septal
branches of the left coronary artery, similar to the Ross oper-
ation. The transection of the ascending aorta just above the sino-
tubular junction may facilitate the root mobilization by
improving the view, especially during the separation of the aortic
root from the pulmonary annulus (Fig. 1).
The main PA will be transected, and the PA confluence will be
brought anterior to the ascending aorta (Lecompte). This maneu-
ver will bring right PA in front of the aorta, thus protecting it
from compression by the posterior movement of the aortic root.
The stenotic LVOT will be opened by longitudinal division of the
anterior commissure of the pulmonary valve extending across
the posteriorly deviated conal septum into the VSD (Fig. 2).
The posterior one-third of aortic annulus will be sewn to the
original pulmonary annulus, and this suture line will be rein-
forced with a second one which approximates the proximal end
of the main PA to the back wall of the aortic root (Fig. 3).
The aortic root will be secured in place of the LVOT, and the
VSD will be closed with the anterior free wall of the RV as a
patch. The distal main PA may be sutured on the new ascending
aorta. This may provide a growing posterior wall for the RV to PA
connection (Fig. 4). If necessary, coronary artery transfer or reim-
plantation may be performed, though the figure does not
show it.
Once the atrial septal defect is closed and the air-tight connec-
tion is made in LVOT, the aortic cross clamp may be removed.
Until this time, if additional cardioplegia injection is needed, it
can be given directly into the bilateral coronary arteries or the
reconstructed aortic root. The final stage will be RV to PA
 H. Nikaidoh / European Journal of Cardio-Thoracic Surgery
Figure 2: Mobilization of the aortic root, division of great arteries, and enlarge-
ment of left ventricular outlet. Ao: ascending aorta; LV: left ventricle; MV: mitral
valve; PA: main pulmonary artery; RA: right atrium; RV: right ventricle; SVC:
superior vena cava; TV: tricuspid valve; VSD: ventricular septal defect.
Figure 3: Implantation of aortic autograft (prior to the Lecompte maneuver). Ao:
ascending aorta; LV: left ventricle; MV: mitral valve; PA: main pulmonary artery;
RA: right atrium; RV: right ventricle; SVC: superior vena cava; TV: tricuspid valve.
connection which can be accomplished with a large gusset of
any material of the surgeon’s choice. I have utilized autogenous
R
pericardium or CorMatrixV (Fig. 5).
1003
EDITORIAL
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Figure 4: Completed aortic root translocation (VSD closed with RV free wall,
anteriorly positioned PA confluence, RA incision for ASD closure). Ao: ascend-
ing aorta; LV: left ventricle; PA: main pulmonary artery; RA: right atrium; RV:
right ventricle; SVC: superior vena cava; TV: tricuspid valve.
In the absence of major postoperative complication, the post-
operative hospital stay will be about 1 week to 10 days.
WHAT DID THIS PROCEDURE ACCOMPLISH?
This procedure proved that the abnormally located AV can be
dependably reimplanted in the LVOT, thus restoring ventriculo-
arterial concordance in various anatomic situations. In addition
to our series [11], Raju et al. [21] reported similar dependability of
LVOT in the latest report from Boston Children’s Hospital in
2015. A similar statement was also made by Kramer et al. [16].
The latest results on RV to PA connection are not ideal, espe-
cially when a valved conduit is utilized [11, 21]. However, in the
absence of a competent pulmonary valve, progressive RV dilation
will lead to the eventual implantation of a prosthetic pulmonary
valve. In theory, later pulmonary valve implantation is considered
better since a larger valve in older patient may last longer. But
this issue has not been fully studied.
A few comparative studies among surgical choices for d-TGA,
VSD and LVOTO are available for Nikaidoh, Rastelli and repar-
ation a l’etage ventricular (REV) operations. However, due to the
retrospective nature of the studies, the case selection, the variable
number in each group, and the relatively short follow-up period,
a definite conclusion is elusive [22–24].
It would be unfair to ignore two special reports among these
choices. Metras et al. [25] reported their modification (autogenous
aortic tissue for RV to PA) of REV operation in 25 patients with
only one reoperation on the RV to PA connection. However, in
three patients, late subaortic stenosis resection was unavoidable.
1004 H. Nikaidoh / European Journal of Cardio-Thoracic Surgery
Figure 5: Completed Nikaidoh procedure (RV–PA connection with a patch).
Ao: ascending aorta; PA: main pulmonary artery; RA: right atrium; SVC: superior
vena cava.
Brown et al. also reported their excellent series of Rastelli oper-
ations on 40 patients, among whom only 2 patients developed
LVOTO and nearly one half needed RV outflow tract reoperation
[26]. Even in these selective experts’ hands, the LVOT remains vul-
nerable in spite of aggressive resection of the conal septum.
However, in 2015, the popularity of the Rastelli operation lin-
gers, as reported by Al-Jughiman et al. [27].
FUTURE
The Nikaidoh procedure has evolved extensively in technical de-
tails and indications described in this article. No procedure re-
mains as it was originally described. The Fontan operation, for
example, has come so far from the original description by Fontan
and Baudet in 1971 [28].
The initial Nikaidoh procedure was done in the days of rather
primitive myocardial protection which presented higher operative
risk. This might have contributed to its slow adoption. Our tech-
nical improvements for performing surgery on the great arteries
and pediatric coronary arteries through ASO have given added as-
surance so that the application of ATL will continue to expand.
However, as we have spent nearly a decade learning the
proper application of ASO, we will continue to search for the
best application of the Nikaidoh principle, i.e. surgical restoration
of venriculoarterial concordance by reimplantation of AV auto-
graft in the LVOT.
Emani et al.’s [23] report from Boston in 2009 included selec-
tion criteria for ATL, though retrospectively, as the initial pulmon-
ary valve Z-score of <-3.
In 2013, the Toronto Children’s Hospital group developed an
anatomic scoring system using 28 patients with d-TGA with
LVOTO for the timing and form of surgical choices such as early
or late ASO, and the Nikaidoh versus Rastelli operation. In the
latter choices, coronary artery distribution appeared to be the
primary determinant [29].
These are the beginnings of an effort to clarify appropriate
choices among all surgical options for the patients with TGA,
VSD, and PS.
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ACKNOWLEDGEMENT
The author is grateful for the illustrations by Rachid Idriss who is
the eldest son of my friend and mentor, the late Farouk S. Idriss.
Conflict of interest: none declared.
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