E31

Adjustment of the Surgical Plan in Repair of Congenital Heart

Disease: The Power of Cross-sectional Imaging and
Three-dimensional Visualization

Rabin Gerrah, MD,* Dianna M.E. Bardo, MD,† Rich D. Reed, PA-C, RN, MPAS,*
Rachel E. Sunstrom, PA-C, MS,* and Stephen M. Langley, MD, FRCS (CTh), FETCS*
*Department of Surgery, Division of Pediatric Cardiac Surgery, Oregon Health and Science University, Doernbecher
Children’s Hospital, and †Department of Radiology, Oregon Health and Science University, Portland, Ore, USA

ABSTRACT

The purpose of this article is to study the importance of cross-sectional imaging in preoperative evaluation and
surgical planning. Echocardiography is the modality of choice to diagnose cardiac diseases. However, in some cases,
the data obtained from echocardiogram are insufficient or the image quality is poor. In these cases, additional
modalities are being used to provide further information that can aid in guiding medical management of or surgical
planning for the patient. Cross-sectional imaging has become widely available in many institutions. These imaging
techniques, especially with three-dimensional reconstructions, provide realistic images that have imperative diag-
nostic values. Moreover, the possibility of sophisticated image-processing techniques provides important hemody-
namic characteristics via less invasive methods. In this article, we present three cases in which additional cross-
sectional imaging seemed to be a crucial step prior to surgical planning.

Key Words. Cross-sectional; Surgery; Three-dimensional; Aortic Arch; Pulmonary Vein

Introduction graphy is not free of limitations and weaknesses.

E chocardiography has become the modality of
choice in the diagnosis of congenital heart
diseases. The noninvasiveness and wide availability
of this diagnostic technique are its greatest benefits,
especially for younger patients. Furthermore,
improvement of ultrasound technology, as well
as additional techniques such as conventional
Doppler methods and Doppler color flow imaging,
has added even more diagnostic power to cardiac
ultrasound for the evaluation of congenital heart
disease. Echocardiography enables us to define the
structure and morphology of the heart, determine
flow directions, measure velocities and gradients,
visualize valves, etc. New probes and image-
processing techniques provide three-dimensional
(3D) images of the heart and its valves.
Currently, echocardiography is the gold stan-
dard in the diagnosis of congenital heart diseases.
In most cases of congenital cardiac disease, surgi-
cal planning and decision making are done based
on echocardiograms only. However, echocardio-
Funding Resources or Grants: None.
© 2013 Wiley Periodicals, Inc.
For instance, lack of appropriate acoustic windows
with specific body habitus or posterior structures
and limited spatial resolution, especially of small
structures, are two of the most frequently encoun-
tered limitations. In some cases, especially when
key anatomy is obscured or inadequately visual-
ized, an additional imaging modality is essential to
provide complete anatomical information.
Cross-sectional imaging modalities such as
magnetic resonance imaging (MRI) and computed
tomographic angiography (CTA) provide an
accurate and comprehensive definition of cardio-
thoracic anatomy with high spatial resolution,
two-dimensional (2D) multiplanar and 3D
surface-rendered reconstructions, and functional
data from 2D planar and four-dimensional or
time-resolved cine angiography. Whether or not
additional imaging modalities should be used and
in which cases has been a controversial issue.
Herein, we present three cases in which cross-
sectional imaging was imperative for preoperative
planning and in determining the course of the
surgery.
Congenit Heart Dis. 2014;9:E31–E36
E32 Gerrah et al.

Figure 1. A low-dose prospectively electrocardiogram-

gated coronary artery computed tomographic angiography
reveals a common origin of the right coronary artery and left
main (LM) coronary arteries from the right sinus of Valsalva
(curved arrow). The LM (black arrow) courses between the
aorta (A) and the pulmonary artery (P). The LM lumen is
round at its origin (white open arrow) but becomes narrow
and oval as it courses in the wall of the aorta (white arrow).

Case 1: Anatomy and Location of the Coronary

Artery in Anomalous Left Coronary Artery

A 14-year-old female was being followed by her

cardiologist for known bicuspid aortic valve. The
patient was asymptomatic. During the follow-up
examinations, an incidental finding of anomalous
left coronary artery arising from the right coronary
sinus was detected by echocardiogram (iE33,
Philips, Best, The Netherlands). This finding war-
ranted full cross-sectional imaging, which included
an MRI (Ingenia, Philips) and CTA (Ingenuity,
Philips) scan. The coronary course and anatomy
were confirmed by these cross-sectional imaging
studies (Figures 1 and 2). The detailed interpreta-
tion of the imaging revealed that the left main
coronary artery arises anomalously from the right
sinus of Valsalva, from a common orifice with the
right coronary artery. The left main coronary
artery has a circular lumen proximally
(4.5 ¥ 3.8 mm), and the lumen becomes oval
(4.3 ¥ 2.4 mm) along a 10-mm segment and then
returns to a circular lumen (4.0 ¥ 3.5 mm) before
bifurcating to the left anterior descending and left
circumflex coronary arteries. These findings are
consistent with an intramural course of the vessel.
In this particular congenital cardiac anomaly, it
is crucial to know whether or not the coronary
Congenit Heart Dis. 2014;9:E31–E36
Figure 2. Three-dimensional surface-rendered recon-
structed images from the coronary artery computed tomo-
graphic angiography reveal the origin of the right coronary
artery and left main coronary arteries from the right sinus of
Valsalva and the normal course of the proximal right coro-
nary artery (R). The left main (white arrows) courses over
the commissure between the right and left leaflets of the
aortic valve (black arrow), between the aorta (A) and the
main pulmonary artery (P).
artery is intramural, as the surgical approach is
quite different. The patient was taken to surgery
and an unroofing of the intramural left main coro-
nary artery was performed. Because the function
of the aortic valve was appropriate, it was pre-
served with no intervention.
The surgical findings were consistent with the
cross-sectional information obtained from the
CTA, which guided surgical planning. The rela-
Utility of Cross-Sectional Imaging in Surgery E33

tionship between the coronary artery and the wall

of the aorta is an important factor in determining
the surgical plan and guiding the decision to
proceed with a coronary unroofing vs. a more
complex procedure involving translocation of the
vessel. In this case, the CTA demonstrated clearly
a large intramural segment and the patient under-
went a successful coronary unroofing.

Case 2: Morphology of the Aortic Arch by

Direct Visualization

A 3-day-old newborn was admitted from an

outside hospital for suspected coarctation of the
aorta. A comprehensive echocardiogram revealed
hypoplasia of the left-sided transverse aortic arch
and discrete narrowing of the isthmus with an
approximately 15-mm Hg gradient and diastolic
flow persistence at the isthmus, consistent with
coarctation. Detailed echocardiogram findings on
the aorta included measurements as follows: aortic
annulus 5.9 mm, z-score -0.9; aortic sinus Figure 3. A three-dimensional surface-rendered recon-
8.8 mm, z-score +3; ascending aorta 7.6 mm, struction of the computed tomographic angiography data
z-score +0.7; transverse aorta 3.5 mm, z-score provides the surgeon a preoperative view of the intratho-
racic anatomy.
-2.6; and aortic isthmus 2.4 mm, z-score -3.7.
Based solely on the initial echocardiogram find-
ings, the appropriate surgical approach would be a
median sternotomy and aortic arch reconstruction
on cardiopulmonary bypass. However, the
serial echocardiograms provided inconsistent data
regarding exact morphology of the aortic arch
without reproducible measurements of each
segment. With some doubt about the exact size
and anatomy of the aortic arch, the patient was
referred for cross-sectional imaging. A cardiotho-
racic CTA study with 3D reconstruction was per-
formed to visualize the arch and depict the
orientation of the brachiocephalic artery branches
(Figure 3). Accurate measurements of the diam-
eter of each aortic segment were performed using
2D images in a direct transaxial plane to the aortic
lumen and were as follows: sinotubular junction
7 mm, ascending aorta 7 mm, aortic arch 3 mm,
isthmus 2 mm, proximal descending aorta 7 mm,
and aorta at the level of diaphragmatic hiatus Figure 4. The aorta is viewed in a sagittal oblique plane
that shows the level of each measurement. Measurements
7 mm (Figure 4).
are made in a transaxial two-dimensional view at each level
While reviewing the anatomic information so that measurements of the lumen are most accurate
from the CTA and directly visualizing the ana- (inset).
tomic reconstruction, it was determined that a left
thoracotomy approach and arch repair with an underwent repair and reconstruction of his aortic
extended end-to-end anastomosis, similar to the arch via a thoracotomy, without cardiopulmonary
repair normally performed for repair of an aortic bypass. The repair was successful and the patient
coarctation, would be possible. Based on the sur- was left with an acceptable 4-mm Hg arm–leg
gical guidance provided from the CTA, the patient pressure gradient.
Congenit Heart Dis. 2014;9:E31–E36
E34
Figure 5. An axial steady-state free precession view of the right atrium shows the right inferior pulmonary vein draining to
the right atrium (open arrow), to the right of the interatrial septum. The margins of a secundum atrial septal defect are marked
(black arrows). The right superior and middle pulmonary veins also drain to the right atrium (vertical right arrows). The
superior vena cava is normal (horizontal white arrow).
Both imaging modalities diagnosed severe
aortic arch hypoplasia. However, the accurate
morphology and visualization of the aorta, espe-
cially in a 3D reconstruction of the CTA, were the
most important factors for decision making in
the surgery. In the presence of inconsistent mea-
surements provided by echocardiogram, the CTA
images provided both a clearer visualization of the
arch and its branches and more consistent mea-
surements, which strongly influenced the decision
to proceed with an extended end-to-end repair in
this patient.
Case 3: Location of the Pulmonary Veins in Partial
Anomalous Pulmonary Venous Return
A 6-year-old male underwent echocardiogram
evaluation after an incidental finding of a murmur
during a routine physical exam. The study showed
a large superior sinus venosus atrial septal defect
with left-to-right shunting. In this echocardio-
gram study, the right-sided pulmonary veins were
not well visualized, and it was conceptualized that
they drained anomalously to the superior vena
cava, given the high superior vena cava flow.
However, this conclusion posed a significant level
of uncertainty, as the veins could not be clearly
visualized on echocardiogram. Therefore, the
patient was referred for further imaging.
An MRI was performed to further define the
anatomy of the atrial septal defect and the exact
location of the right-sided pulmonary venous con-
nection to the heart. While the echocardiogram
Congenit Heart Dis. 2014;9:E31–E36
Gerrah et al.
suggested the veins connected to the superior vena
cava, the MRI study clearly showed that the right
upper and right lower pulmonary veins connected
completely to the right atrium and to the atrial
septal defect located in the superior portion of the
septum (Figure 5).
The information from the MRI study was used
in the presurgical planning for closure of the septal
defect in this patient. Specifically, we were able to
visualize exactly where the veins came into the
heart and plan the exact patch location to redirect
the veins to the correct side of the heart. This
anomaly was repaired with partial resection of the
native atrial septum and closure of the defect with
a patch that was sutured above the level of the
pulmonary veins, thereby directing the pulmonary
venous flow to the left atrium.
Discussion
Over the past two decades, echocardiography
techniques have been optimized and are consid-
ered an ideal diagnostic tool for congenital heart
diseases.1 Echocardiography has become the gold
standard in this field. However, it continues to
have some weaknesses with spatial resolution
and acoustic windows. Cross-sectional imaging
techniques, including CTA and MRI with sophis-
ticated image-processing techniques, have over-
come these limitations.2,3
MRI and CTA imaging techniques provide
high-quality cross-sectional images in any desired
plane. Three-dimensional image acquisition in
Utility of Cross-Sectional Imaging in Surgery
both imaging modalities allows repeated and infi-
nite image processing following the examination
and is especially helpful for direct consultation
with the radiologist when nontraditional views of
the heart and cardiothoracic anatomy are required.
Both imaging modalities can provide reliable and
accurate data, especially when there remains any
level of uncertainty in reviewing more traditional
echocardiography data.
Different imaging modalities have been com-
pared for evaluation of the anomalous coronary
arteries in adults.4 However, these guides are less
applicable in small patients and children. Despite
echocardiography’s strength in diagnosing this
anomaly by using color Doppler flow mapping,5
some doubt remains regarding the course of the
vessel. In coronary anomalies, CTA provides high-
quality images with stunning spatial resolution and
unsurpassed image reconstructions, delineating
the coronary course.
In the case of aortic arch repair, accurate
measurements were available from both the
echocardiogram and CTA. However, the 3D
CTA images in a 360° rotation allowed us to vir-
tually plan the anastomosis lines and the extent
and specific angle of excision. This complete
visualization is achieved by using off-axis image
planes to easily increase accuracy of vessel lumen
and chamber measurements and using those same
variable planes to further improve understanding
of presurgical anatomy. These advantages are
lacking when relying upon the standard planes of
echocardiography.
Another advantage of the cross-sectional
images is the ability to delineate adjacent tissues
and organs that are often unchecked or not evalu-
able with echocardiography, as demonstrated in
the third case. Because the pulmonary veins lie at
the interface between the acoustically friendly
heart and the air-filled, nonacoustic lungs, their
anatomic definition by echocardiography is often
inadequate. With cardiac CTA and MRI, the
anatomy of the pulmonary veins, and specifically
their location in relation to the interatrial septum,
is determined. This information is a crucial point
in presurgical planning. The application of cardiac
MRI in planning of pulmonary vein redirection in
this setting has been studied previously.6
The cases presented here exemplify the ben-
efits of cross-sectional imaging in situations
where echocardiogram is limited or is unable to
provide complete information. Adding a second
imaging modality such as cardiac MRI or CTA to
echocardiogram provides crucial anatomic and
E35
physiologic information for improved under-
standing and surgical planning. The similarity of
reconstructed MRI and CTA images to real-life
intraoperative views of cardiovascular anatomy
facilitates the conceptualization, imagination, and
planning of the surgical repair prior to opening
the chest.
Based on lessons learned from these and numer-
ous other cases, we recommend performing
cross-sectional imaging in addition to routine
echocardiography in patients where there remains
some level of uncertainty. The need for adding
another imaging modality and its potential risks,
such as exposure to radiation, sedation, or contrast
material, must be considered in each individual
case as part of a risk/benefit calculation.
Author Contributions
Rabin Gerrah—Concept, data approval and literature
search, first draft.
Dianna M.E. Bardo—Data analysis and interpretation of
the images.
Rich D. Reed—Draft and revision of the article.
Rachel E. Sunstrom—Collection of the data, draft and
revision of the article.
Stephen M. Langley—Critical revision of the article.
Corresponding Author: Rabin Gerrah, MD, Pediatric
Cardiac Surgery, Oregon Health and Science Univer-
sity, Doernbecher Children’s Hospital, 3181 SW
Sam Jackson Park Road, DC-8S, Portland, OR 97239,
USA. Tel: 503-418-5443; Fax: 503-418-1385; E-mail:
gerrah@ohsu.edu
Conflict of interest: None.
Accepted in final form: February 1, 2013.
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