1 s2.0 S1934592522000673 Main

Journal of Cardiovascular Computed Tomography 16 (2022) 467–482
Contents lists available at ScienceDirect
Journal of Cardiovascular Computed Tomography

journal homepage: www.JournalofCardiovascularCT.com
State of the Art in Cardiovascular CT’
Cardiovascular computed tomography in pediatric congenital heart disease:

A state of the art review
Jennifer Cohen a, Priyanka Asrani b, Simon Lee c, Donald Frush d, B. Kelly Han e,
Anjali Chelliah b, f, Kanwal M. Farooqi b, *
a
Department of Pediatrics, Division of Pediatric Cardiology, Icahn School of Medicine at Mount Sinai, New York, NY, USA
b
Division of Pediatric Cardiology, Department of Pediatrics, New York Presbyterian/Columbia University Irving Medical Center, New York, NY, USA
c
Department of Pediatrics, The Heart Center, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA
d
Division of Pediatric Radiology, Department of Radiology, Medical Physics Graduate Program, Duke University Medical Center, Durham, NC, USA
e
The Children's Heart Clinic at The Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, USA
f
Division of Pediatric Cardiology, Goryeb Children's Hospital, Atlantic Health System, Morristown, NJ, USA
A R T I C L E I N F O A B S T R A C T
Keywords: Cardiac computed tomography (CCT) has increasingly been used in the assessment of both children and adults
Cardiac computed tomography with congenital heart disease (CHD), in part due to advances in CCT technology and an increased prevalence of
Congenital heart disease adults with palliated CHD. It serves as a complimentary modality to echocardiography, cardiac magnetic reso-
nance imaging and cardiac catheterization. CCT can provide unique diagnostic information, is less invasive and
less likely to require sedation compared to other modalities. Detailed knowledge of individual patient cardiac
anatomy, physiology, surgical repair and possible residual lesions are paramount to optimal CCT imaging. This
comprehensive review details the use of CCT both pre- and postoperatively for the most common CHD diagnoses.
We also aim to highlight some new and innovative technologies that have become available and can further
optimize CCT imaging for CHD patients.
guidelines have been published that detail the use of CCT in CHD,
including the rationale, utility and technical recommendations.3,4 In this
1. Introduction
comprehensive review, we focus on the most common indications for
CCT in CHD and detail practical considerations for performance for both
Congenital heart disease (CHD) remains the most common type of
pre- and post-operative assessment.
major congenital malformation affecting 0.8%–1.2% of livebirths
worldwide.1 Cardiovascular imaging plays a significant role in initial
2. Technique
evaluation, management planning and long term follow up for these
patients. Although echocardiography continues to be the workhorse of
2.1. Sedation
noninvasive imaging for patients with CHD, advanced imaging modal-
ities are utilized in numerous scenarios in which additional diagnostic
The need for sedation during CCT depends on the age and develop-
detail is needed, often in the setting of poor image quality, due to limited
mental level of the patient, the anatomy of interest as well as the type of
echocardiographic windows.2 Cardiovascular computed tomography
scanner being used. For infants less than 4 months of age, the “feed and
(CCT) offers the advantage of submillimeter isotropic spatial resolution
swaddle” technique can often be utilized. Sedation may still be required
with short scan times and image quality not limited by ferromagnetic
to minimize motion, slow the heart rate and control respirations if
artifact in patients with prior interventions.3,4 CCT in infants with CHD
detailed evaluation is needed of very small structures such as coronary
has often been shown to add diagnostic information and impact man-
arteries or severely hypoplastic branch pulmonary arteries. Patients be-
agement.5 Development of innovative dose optimization techniques al-
tween 4 months and approximately 6 years of age may require sedation
lows minimization of radiation exposure in a population which is more
depending on the type of scanner being used. The improved temporal
sensitive to ionizing radiation than their adult counterparts.6 Prior
* Corresponding author. New York Presbyterian/Columbia University Irving Medical Center, Morgan Stanley Children's Hospital, 3959 Broadway, CHN 2, New
York, NY 10032, USA.
E-mail address: kf2549@cumc.columbia.edu (K.M. Farooqi).
https://doi.org/10.1016/j.jcct.2022.04.004
Received 11 November 2021; Received in revised form 27 April 2022; Accepted 28 April 2022
Available online 6 May 2022
1934-5925/© 2022 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.
J. Cohen et al. Journal of Cardiovascular Computed Tomography 16 (2022) 467–482
Abbreviations MRI magnetic resonance imaging

PA pulmonary artery
AAOCA anomalous aortic origin of the coronary artery PAPVC partial anomalous pulmonary venous connection
ASO arterial switch operation RV right ventricle
CCT cardiac computed tomography RVOT right ventricular outflow tract
CHD congenital heart disease SCD sudden cardiac death
DORV double outlet right ventricle SVC superior vena cava
HU Hounsfield unit TAPVC total anomalous pulmonary venous connection
ICD implantable Cardioverter defibrillators TGA transposition of the great arteries
IV intravenous TOF tetralogy of Fallot
IVC inferior vena cava TOF/PA tetralogy of Fallot with pulmonary atresia
kV kilovoltage VSD ventricular septal defect
LV left ventricle 3D three-dimensional
mA milliampere 4D four-dimensional
MAPCAs major aortopulmonary collateral arteries
resolution of dual source scanners and increased z-axis coverage of wide- 4. Bolus tracking
detector scanners allows scans to be performed within a single heartbeat,
which often obviates the need for sedation. Vacuum immobilization When bolus tracking is used, the image acquisition is automatically
devices may be helpful to reduce motion during the scan.7 Older gener- triggered when the contrast reaches a set Hounsfield unit (HU) in the
ation scanners with fewer detector rows acquire images over multiple defined structure of interest. Alternatively, manual bolus tracking in-
heart beats and are more likely to require sedation or a breath hold for volves manually triggering the scan during real-time visualization of
optimal imaging.4,8,9 contrast in the area of interest on the monitoring sequence. Automatic
bolus tracking works best for older (adolescent or adult) patients who are
3. IV and contrast injection protocol less likely to move during the scan and those with normal venous and
arterial connections and minimal shunting. For smaller children and
To optimize contrast injection, the patient care team should aim to those with more complex cardiac disease, manually triggering the scan is
place the largest peripheral intravenous (IV) line possible. Twenty-four often more successful in yielding optimal contrast opacification. In
gauge IVs are typically placed in infants with a maximal flow rate of 1.5 complex CHD, placing the monitoring slice in the mid-heart with
mL/s, though fenestrated IV catheters have been shown to allow a faster simultaneous visualization of the descending aorta can be helpful to
flow rate.10,11 IV access location should be chosen based on the anatomy of optimize scan timing, though monitoring at the upper mediastinum may
interest. For example, injection in a lower extremity vein (or contrast be adequate in some cases. A test bolus (or timing bolus) can also be used
dilution with saline with an upper extremity site) may be beneficial in in complex cases.
neonates to avoid “streak artifact” from beam hardening in the superior
vena cava (SVC) which may obscure adjacent structures such as the right 5. ALARA and scan parameters
pulmonary artery. Iodine based contrast is utilized for image acquisition
with the volume administered determined by a weight based calculation, As with all imaging modalities that use ionizing radiation, CCT in
taking into account the maximal IV flow rate and anatomy of interest. CHD must abide by the “as low as reasonably achievable” (ALARA)
Injection protocols will depend on the structures of interest. concept and minimize the radiation used to obtain the necessary
Frequently used protocols in imaging CHD include a biphasic (dual- diagnostic information. The scan length should be carefully prescribed
phase) injection, triphasic (biventricular) injection and a venous two- to include only those structures needed to answer the clinical question.
phase injection4 (Table 1). A biphasic (dual-phase) injection involves
injecting contrast at a constant rate followed by a saline flush. This is
Table 1
typically used when the structures of interest are either the pulmonary or
Example of contrast injection protocols with an example given for a 10 kg child
systemic arterial vasculature (not both). When there is significant
with a 22G IV. Exact injection protocol will be tailored to the patient anatomy
intra-cardiac mixing this protocol can also be used to visualize both the and physiology and structure of interest (can be determined by multiplying
right and left sides of the heart with a slower injection. maximum IV flow rate by how many seconds of contrast opacification you want).
A triphasic (or biventricular) injection is used for simultaneous 1Can substitute with contrast and run at a slower rate than the first contrast
visualization of the right and left heart structures (pulmonary and sys- injection. 2Can also perform a biphasic scan with a 30–60 s delay to trigger the
temic arterial vasculature). This can be done by giving one-half to two- scan. 3Timing of pause can be assessed with a timing bolus and depends on
thirds of contrast at the usual rate for IV size and then the remainder patient size, heart rate, cardiac output and structures of interest.
at a slower rate, followed by saline flush. Another option is to mix the Injection rate Volume Time of injection
second phase of the contrast bolus as 50:50 saline and contrast and keep (mL/s) (mL) (s)
the rate constant. Biphasic (dual phase) injection
A venous two-phase injection (or delayed scan) is used when visual- Contrast 2.5 mL/s 18 mL 7s
ization of both the systemic venous and arterial anatomy are needed, Saline 2.5 mL/s 10 mL 4s
mostly commonly in patients with single ventricle anatomy after a Triphasic (biventricular) injection
Contrast 3.0 mL/s 12 mL 4s
bidirectional Glenn anastomosis or Fontan palliation. An initial acquisi-
Contrast/Saline 50:50 mix1 3.0 mL/s 8 mL 2.7 s
tion captures the systemic arterial anatomy well, while a delayed Saline 2.0 mL/s 10 mL 5s
acquisition opacifies the systemic venous pathways including the Glenn Venous 2-phase injection2
and Fontan. Simultaneous injection in an upper and lower extremity IV Contrast 2.5 mL/s 9 mL 3.6s
Pause variable3
has also been employed in an effort to uniformly opacify the systemic
Contrast 3.0 mL/s 11 mL 3.7s
venous anatomy in the initial acquisition. However, delayed scanning Saline 3.0 mL/s 10 mL 3.3s
with a single site injection appears to give the best results.12,13
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Scanner voltage (kV) and tube current (mA) should be set as low as 3D imaging dataset is processed by a 3D printer to create a physical
considered reasonable, with 70 kV often being adequate in neonates model. Cardiac models are utilized for pre-surgical planning, preparation
and infants and 80 kV in older children and teenagers. Low kV scan- for interventional procedures and teaching of patients and trainees.17–21
ning can also aid in achieving higher contrast brightness. Higher kV CCT is an optimal imaging modality for 3D printing due to the excellent
may be needed in those with metallic implants (such as stents or spatial resolution and isotropic dataset obtained. The scan parameters
prosthetic valves) to reduce metal artifact, though projection-based used to optimize images for 3D model creation should focus on opacifi-
metal artifact reduction algorithms can also be used when avail- cation of the region of interest with contrast, with the goal of a clear
able.14,15 The lowest mA should be used based on the kV selected and delineation between the structures of interest and extraneous
the option of automatic tube current modulation should be utilized if structures.22,23
available. When gating is necessary, most scans for CHD can be pro-
spectively gated.16 Retrospective gating should be reserved for sce- 7. Specific cardiac lesions
narios in which cine imaging is needed such as assessment of
ventricular size and function or when cardiac magnetic resonance 7.1. Nomenclature in congenital heart disease
imaging (MRI) is unable to be performed.4 Dose modulation should be
used if retrospective gating is performed. Detailed understanding of cardiac anatomy and physiology is essen-
tial to optimization of performing CCT in patients with CHD. Nomen-
6. 3D printing clature in this group of patients is particularly important to ensure proper
communication of findings. A segmental approach to CHD includes
Three dimensional (3D) printing is a technology that is increasingly assessment of each cardiac segment independently as well as the
being applied to cardiovascular modeling. A digital model created from a connection between segments. Thoracic and abdominal situs (or
Fig. 1. (A–C). Cardiac computed tomography images of a teenager with history of D-transposition of the great arteries (TGA) s/p arterial switch operation (ASO), with
a single right coronary artery. (A) Axial image in which branch pulmonary arteries are well seen s/p Lecompte maneuver with the branch pulmonary arteries draped
around the ascending aorta (*). (B). Axial oblique image demonstrating a single right coronary artery (arrow) with the left coronary artery coursing anterior to the
pulmonary artery (*). (C). 3D volume rendering demonstrating branch pulmonary arteries anterior to the aorta and single coronary artery is seen with left coronary
coursing anterior to the pulmonary artery (arrow) (D). Axial oblique image in a teenage patient also with D-TGA s/p ASO and typical coronary orientation with the
right coronary artery reimplanted into the right facing sinus and left coronary into the left facing sinus. The main pulmonary artery (*) is anterior to the aorta.
469
sidedness) should be determined first to give an overall anatomic the pulmonary artery anterior to the aorta such that the branch pulmo-
framework. Situs solitus refers to the usual arrangement of lateralized nary arteries flank the ascending aorta.30
abdominal organs and lung anatomy, situs inversus is the mirror image of Pre-operatively, CCT is rarely performed for patient with D-TGA as
normal, and situs ambiguous (heterotaxy) refers to abnormal symmetry anatomic details can typically be delineated by echocardiography.
and inconsistent abdominal and thoracic organ lateralization.24 The Postoperatively, complications that can occur after ASO include coronary
“building blocks” of the heart are the veins and atria, ventricles and great ostial stenosis, neo-pulmonary artery and branch pulmonary artery ste-
arteries. A normal heart will have atrioventricular and ventriculoarterial nosis, neo-aortic root dilation or supra-aortic stenosis. The anterior
concordance, with a sub-pulmonary conus connecting the right ventricle location of the main and branch pulmonary arteries after the Lecompte
to the main pulmonary artery. Conal septum is the region of the conus maneuver can make them particularly difficult to visualize well on
between the aorta and main pulmonary artery and this can be deviated in echocardiography and advanced imaging is often needed for further
malalignment ventricular septal defects (VSD). evaluation. Although cardiac MRI is often the advanced imaging mo-
dality of choice for routine postoperative assessment in these patients, in
8. Transposition of the great arteries patients with contraindication to MRI (such as the presence of pace-
makers, implantable cardioverter defibrillators (ICD), significant artifact
D-Transposition of the great arteries (D-TGA) is a CHD with ven- on MRI or claustrophobia), CCT provides high-resolution imaging to
triculoarterial discordance, whereby the aorta arises from the right assess for any residual lesions.31 CCT is the modality of choice in
ventricle and the pulmonary artery arises from the left ventricle. The assessment of coronary arteries as well as assessment of branch pulmo-
incidence is 31.5 in 100,000 live births and it is the second most common nary arteries if stents are present. Coronary artery assessment is recom-
cyanotic CHD.25 D-TGA can occur in isolation or it may be associated mended in all symptomatic patients after ASO and during adolescence or
with other cardiac anomalies. Associated cardiac defects include simple early adulthood in asymptomatic patients.32,33 Fig. 1A–C demonstrates
VSDs or more complex malalignment VSDs with associated outflow tract CCT images in a teenage patient with history of D-TGA and a single right
obstruction.26 coronary who has undergone an ASO. Fig. 1D demonstrates coronary
The atrial switch operation, initially performed in the 1950s, involved imaging in a different teenage patient after ASO with typical coronary
the systemic venous blood flow being redirected to the mitral valve and artery origins.
the pulmonary venous blood redirected to the tricuspid valve.27 This was Complications that can occur after atrial switch include baffle
a “physiologic” correction, however it left the RV pumping to the sys- obstruction or leak, systemic RV failure, tricuspid regurgitation and sub-
temic circulation which has resulted in long term complications of right pulmonary obstruction (due to ventricular septal shift). Many of these
ventricular failure and tricuspid regurgitation.28 The arterial switch patients have pacemakers or ICDs, rendering CCT the imaging modality
operation (ASO) with coronary reimplantation was first successfully of choice to assess for postoperative complications. Baffle obstruction can
performed in 1975 and has become the standard repair approach.29 As accurately be assessed on CCT. There may be streak artifact in the SVC
part of the ASO, the LeCompte maneuver is performed which relocates from contrast injection limiting assessment of the superior limb of the
Fig. 2. (A–C). Cardiac computed tomography in an adult patient with h/o D-transposition of the great arteries (TGA) s/p atrial switch. (A) Axial image in which the
aorta (*) is seen anterior and rightward to pulmonary artery. The branch pulmonary arteries are dilated. (B). The pulmonary venous baffle (arrow) is well seen and is
unobstructed. (C). The superior vena cava baffle (dashed line) is seen in an oblique coronal view with evaluation of baffle limited by streak artifact from contrast.
(D–F). Adult patient with h/o D-TGA, ventricular septal defect (VSD) and pulmonary stenosis s/p Rastelli repair with Melody valve in the right ventricle (RV) to
pulmonary artery (PA) conduit. (D,E). The pathway from the left ventricle to the aorta through the VSD is seen (dashed arrow) and is slightly small particularly in the
right-left dimension. The aorta is dilated. (F). The RV to PA conduit is well demonstrated with some artifact from the stent of the Melody valve.
470
systemic baffle however this can be mitigated by performing a delayed aneurysms. CCT also provides excellent spatial resolution allowing
scan, in which the inferior vena cava (IVC) limb of the baffle can also be assessment the relationship between the RV to PA conduit, coronary
seen. The pulmonary venous baffle is often the easiest baffle to visualize arteries and the sternum. CCT is often the modality of choice to assess the
on CCT (Fig. 2A–C). Ventricular function can also be assessed if retro- right ventricular outflow tract in anticipation of trans-catheter pulmonary
spective gating is used. Baffle leaks are often difficult to assess on CCT. A valve placement. The pathway from the LV to the aorta can also be
baffle leak would best be seen as a negative or positive contrast jet, assessed (Fig. 2D–F). Prospective gating can be used if only anatomy is
however when a biventricular injection is used both atria will be opaci- being assessed. However if the scan is being performed to assess for
fied similarly, making this difficult to detect. candidacy for transcatheter pulmonary valve placement, then a retro-
Patients with more complex variants of D-TGA such as D-TGA with spective scan may be needed to assess the right ventricular outflow tract
VSD and left ventricular outflow tract obstruction may require a more size and relationship to the coronaries throughout the cardiac cycle
complex repair such as the Rastelli operation (baffling the left ventricle depending on the anatomy of the RVOT and type of transcatheter valve
(LV) to the anterior aorta with closure of the VSD and placement of a RV under consideration.35,36
to pulmonary artery (PA) conduit).34 The Nikaidoh procedure, which
involves aortic root translocation, may also be performed. Postoperative 9. Tetralogy of Fallot
complications include RV to PA conduit obstruction or obstruction to the
pathway from the LV to the aorta. These can be assessed by echocar- Tetralogy of Fallot (TOF) is the most common form of cyanotic CHD,
diogram in the younger population and by cardiac MRI when echocar- accounting for 4 to 6% of CHD and occurs in about 1 in 3000 live
diographic windows are suboptimal. RV to PA conduits are often quite births.37,38 TOF is characterized by anterior deviation of the conal ven-
anterior rendering them difficult to visualize on echocardiogram in older tricular septum associated with an anterior malalignment ventricular
patients. In patients with contraindications to MRI, CCT is used to assess septal defect, aortic override above the ventricular septum, and pulmo-
RV to PA conduit anatomy and stenosis, calcification or any associated nary outflow tract obstruction. The degree of right ventricular outflow
Fig. 3. Cardiac computed tomography in a 3 day old patient with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs).
There was no evidence of a main pulmonary artery segment. (A) A coronal oblique view demonstrates the malalignment ventricular septal defect and overriding aorta
(Ao). (B) Maximal intensity projection image demonstrates the origin of the right and left MAPCAs (red arrows) arising from the proximal descending aorta. A 3D
volume rendering of the whole heart allows visualization of the right MAPCA from the posterior and rightward aspect (C) and the left MAPCA viewed anteriorly (D).
Left ventricle (LV), right ventricle (RV). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
471
Fig. 4. Axial (A) and Sagittal (B) Cardiac CT images in a neonate with cardiac dextroposition (due to ball-valve air trapping of the left lung causing left lung hyperinflation)
and absent pulmonary valve syndrome demonstrating severe narrowing of the left mainstem bronchus (red arrow) secondary to severe pulmonary artery (PA) dilation.
Aorta (Ao), right ventricle (RV). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
tract obstruction is highly variable, ranging from minimal subpulmonic Fetal and postnatal echocardiography are the mainstay of diagnostic
stenosis to pulmonary atresia (TOF/PA). In its most severe form, severely imaging in TOF. In recent years, CCT has emerged as a noninvasive
hypoplastic or absent central pulmonary arteries are replaced by major alternative to diagnostic cardiac catheterizations that were previously
aortopulmonary collateral arteries (MAPCAs) arising from the aortic arch. performed in many TOF patients to better delineate anatomic details and
Fig. 5. Cardiac computed tomography images of an infant with double outlet right ventricle. (A). The aorta and pulmonary artery both arise from the right ventricle
(RV) and are nearly side-by-side with a rightward aorta (Ao). (B). The RV and left ventricle (LV) are normal sized and the ventricular septal defect (VSD) is moderate
sized with extension into the inlet septum. (C). The position of the Ao and RV are shown from a sagittal plane. (D). A coronal image demonstrates the spatial
relationship of the LV, VSD and Ao and a relatively long potential baffle pathway (dashed arrow).
472
guide intervention. The high spatial and temporal resolution conferred and less radiation or anesthesia exposure time, though limited data has
by CCT makes it particularly useful in imaging infants with unrepaired not demonstrated a significant difference.47
TOF. About 5% of patients with TOF have abnormal coronary artery CCT is also used to guide management of TOF with absent pulmonary
anatomy, including an accessory left anterior descending coronary artery valve, a TOF variant with a rudimentary pulmonary valve causing free
or prominent conal branch that crosses the right ventricular outflow pulmonary insufficiency in utero and massive dilation of the pulmonary
tract, which can complicate neonatal TOF repair.39,40 Echocardiographic arteries.48 Pulmonary artery enlargement leads to airway compression
imaging of coronary anatomy is often limited. However, newer CCT and a “ball-valve” mechanism of fluid or air trapping that often results in
technology has made it feasible to image coronary anomalies in young a critically ill postnatal presentation. CCT can delineate the tortuous and
children with TOF with upwards of 97% sensitivity compared to invasive dilated pulmonary arteries, as well as areas of associated tracheobron-
angiography and intraoperative findings.41,42 chial compression and parenchymal changes, in order to plan surgical
In neonates with TOF undergoing staged repairs, CT angiography has repair (Fig. 4).49
also been used to guide transcatheter procedures, such as stenting of the Surveillance of patients after TOF repair is determined based on
RV outflow tract or patent ductus arteriosus, and to identify optimal symptoms and associated residual lesions. For postoperative monitoring
vascular access routes.43 In TOF patients who are initially palliated with in these patients, CCT imaging can often overcome the imaging limita-
stents or surgical aortopulmonary shunts, CCT is often obtained prior to tions of echocardiography and even cardiac MRI. CT angiography is used
full repair to evaluate shunt patency and assess for pulmonary artery to assess RV to PA conduit calcification and obstruction, right ventricular
stenoses or aneurysms. outflow tract (RVOT) pseudoaneurysms, and pulmonary artery stenosis.
In patients with TOF/PA and MAPCAs, surgical management involves In patients with non-MRI compatible pacemakers, retrospectively gated
mobilizing the tortuous network of aortopulmonary collaterals from the CCT has been used to assess biventricular volume and function with good
aorta and incorporating them into the pulmonary outflow. Convention- correlation with MRI measurements.50,51 When subsequent surgical in-
ally, invasive catheter-based angiography was performed at birth to terventions are performed, CCT can identify areas of potential adhesion
delineate the pulmonary arteries and MAPCAs and identify the source of between the RVOT and the sternum to minimize the risk of vessel injury
blood supply to each lung segment. At many institutions, CCT is now a during repeat sternotomy.52
first-line noninvasive, relatively low-radiation imaging option in neo- CCT imaging also plays a key role in assessing candidacy for percu-
nates that can often be performed without sedation (Fig. 3). While the taneous pulmonary valve replacement in TOF patients and others with
spatial resolution of CCT is slightly inferior to that of catheter-based RV to PA conduits, such as after truncus arteriosus repair or pulmonary
angiography, it provides excellent visualization of the pulmonary ar- autograft of the aortic valve (Ross operation); preprocedural measure-
teries and MAPCAs as well as the relationship of each collateral to the ment protocols are becoming increasingly standardized and vali-
airway and esophagus.44–46 Preoperative catheterization may be delayed dated.35,53 Preprocedural CCT is also recommended to identify coronary
until the patient is older and at less risk of catheter-related complications arteries in proximity to the outflow tract that may be compressed by a
such as vessel injury and can focus on identifying areas of collateral stent, a contraindication to transcatheter valve replacement.54 During
stenosis and the blood supply to each lung segment. Guidance from early percutaneous pulmonary valve replacement, CCT datasets can be
CCT imaging could also potentially allow for fewer invasive angiograms co-registered with live fluoroscopy to provide real-time procedural
Fig. 6. A 3D printed model created from the cardiac computed tomography images which was cropped to represent the superior (sup) and inferior (inf) cardiac
anatomy is shown. The great arteries are side-by-side with the aorta (Ao) rightward of the pulmonary artery (PA) and both arising from the right ventricle (RV). The
path from the left ventricle (LV) to the aorta (Ao) through the ventricular septal defect (VSD) is delineated (dashed arrow).
473
Fig. 7. Cardiac computed tomography imaging of unobstructed infracardiac total anomalous pulmonary venous connection. (A) Coronal MIP viewed from the anterior
aspect and (B) 3D reconstruction imaging viewed from the posterior aspect showing all the pulmonary veins coming together to form a confluence (*) which descends
vertically and connects to the inferior vena cava.
guidance.55 Patients with transcatheter pulmonary valves are at relationships using an advanced imaging modality offers essential
increased risk of infective endocarditis that often cannot be easily anatomic information. CCT is commonly applied for this purpose and
assessed by echocardiogram.56 CCT has been used to diagnose percuta- allows for a more complete pre-surgical assessment of the intracardiac
neous valve-associated infective endocarditis by identifying in-stent de- anatomy. The images from CCT can also be post-processed to create a 3D
fects representing thrombus and vegetation.57 whole heart virtual or printed model to help plan a potential left ventricle
to aorta baffle. The need for delineation of this anatomy has led to DORV
10. Double outlet right ventricle becoming somewhat of the prototypical CHD for which 3D models from
CCT are created.17,59
Double outlet right ventricle (DORV) is a form of CHD characterized The CCT images of an infant with DORV with a remote VSD and
by both the pulmonary artery and aorta arising from the right ventricle, pulmonary stenosis are presented in Fig. 5. The patient's anatomy con-
most commonly associated with a VSD. It is an uncommon CHD with an sisted of two normal sized ventricles and a moderate sized VSD. The
estimated incidence of 0.13 per 1000 live births.25 The position of the images demonstrated that the moderate sized VSD was remote from both
VSD in relation to the great arteries is categorized as subaortic, sub- the aorta and the pulmonary artery resulting in a long potential baffle
pulmonary, doubly committed, or remote. The VSD position and degree pathway from the left ventricle (LV) through the VSD to the aorta
of outflow tract obstruction largely determine the physiology of this (Fig. 5D). Based on the patient's echocardiogram, CCT images and clin-
lesion. Clinical presentation can range anywhere from pulmonary ical status, he underwent a bidirectional Glenn anastomosis between the
over-circulation to cyanosis due to mixing and limited pulmonary blood SVC and the right pulmonary artery, to allow growth prior to complete
flow. In patients in whom the cardiac anatomy is amenable, a definitive repair. A 3D printed cardiac model was created to allow direct visuali-
two ventricle repair involves placement of a baffle, routing blood flow zation of the 3D cardiac anatomy for definitive repair (Fig. 6). The patient
from the left ventricle to the aorta. When such a repair is not possible, underwent a Nikaidoh procedure which involved aortic root trans-
patients may have to undergo a multi-stage palliative surgery, culmi- location with VSD closure and RV to PA conduit placement at 2.5 years of
nating in the single ventricle “Fontan” circulation.58 age. CCT has a central role in outlining the cardiac anatomic relation-
Although the initial diagnosis for patients with DORV is based on ships in patients with DORV and allows more comprehensive consider-
echocardiographic images, further delineation of the complex 3D ation of surgical options in this complex CHD.
Fig. 8. (A) Coronal cardiac computed tomography image is viewed from the anterior aspect showing partially anomalous pulmonary venous connection of a right
upper and a right middle pulmonary vein (marked with arrows) draining to the SVC. (B) An axial CT image of an associated superior sinus venosus atrial septal defect
(*) is shown.
474
Fig. 9. 3D reconstruction of the pulmonary veins in a former premature (28 week) infant with right and left-sided pulmonary veins stenosis (A) before surgery. The
patient developed recurrent pulmonary vein stenosis approximately 6 months after sutureless repair (B) with moderate to severe stenosis of the left-sided veins and
near atresia of the right upper pulmonary vein (arrow).
11. Pulmonary venous anomalies and sinus venosus atrial septal infradiaphragmatic course. The development of obstruction is usually an
defect indication for immediate surgical intervention.62,63 Partial anomalous
pulmonary venous connection (PAPVC) implies that either one or more
Pulmonary venous anomalies consist of a group of congenital lesions pulmonary veins connect or drain abnormally with at least one other
in which some or all of the pulmonary veins either connect or drain pulmonary vein connecting normally to the left atrium. This can be seen in
anomalously to the systemic veins or the right atrium directly. Total association with a sinus venosus atrial septal defect, which can be superior
anomalous pulmonary venous connection or drainage (TAPVC) occurs (most commonly) or inferior. A superior sinus venosus atrial septal defect
when all of the pulmonary veins connect or drain abnormally, with a refers to an interatrial communication often including a deficiency of the
prevalence of 1–5% of all CHD.60 An atrial septal defect is the most common wall between the SVC and the right sided pulmonary veins.64
commonly associated lesion with anomalous pulmonary veins. Scimitar syndrome refers to a condition of PAPVC of the right sided pul-
TAPVC can be further classified into 4 categories based on the monary veins usually to the IVC. This is associated with varying degrees of
anatomic site of drainage (Darling classification).61 other cardiovascular and pulmonary anomalies including right lung and
right pulmonary artery hypoplasia with the possibility of systemic arterial
a. Supracardiac (most common) – connection to the innominate vein, supply to the lung and lung sequestration.65
SVC, or right atrium typically via a vertical vein. Primary pulmonary vein stenosis is a rare entity and has been described
b. Cardiac – connection to the coronary sinus. in association with prematurity, particularly in those with chronic lung
c. Infracardiac – connection to the IVC, hepatic vein, or portal vein. disease. It has relatively poor outcomes despite interventions, with the
d. Mixed – combination of any of the above. development of pulmonary arterial hypertension with progressive dis-
ease.66,67 Secondary pulmonary vein stenosis in pediatric patients occurs
All types of TAPVC are at risk for developing obstruction, however, it is most commonly postoperatively in ~10% patients after repair of TAPVC.
most commonly seen with infracardiac type of TAPVC typically due its Pulmonary vein stenosis can appear discrete, as a longer segment of
Fig. 10. Cardiac computed tomography in a 2 year old patient with mesocardia, hypoplastic left heart syndrome, mitral and aortic atresia, bilateral superior vena
cavae, status post Norwood and subsequent bilateral bidirectional Glenn anastomoses. A. Axial image of the single right ventricle (RV) demonstrating unobstructed
pulmonary venous drainage (*) into the left atrium and a large atrial communication after atrial septectomy. B. Axial oblique image demonstrating the Damus Kaye
Stansel anastomosis between the native aorta (Ao) and the native pulmonary artery or neoaorta (*). C. A 3D Volume rendering viewed from the posterior aspect with
visualization of the bilateral bidirectional Glenn anastomoses. Left and right bidirectional Glenn (LBDG, RBDG).
475
Fig. 11. Cardiac computed tomography images in a 9 year old patient with hypoplastic left heart syndrome, mitral and aortic atresia s/p extracardiac fenestrated
Fontan palliation. Oblique axial (A,D) coronal (B,E) and sagittal (C,F) images demonstrate the utility of an initial acquisition (A–C) which provides visualization of the
bidirectional Glenn (out of plane), pulmonary arteries (PA), systemic ventricle (SV) and systemic arteries (SA). The delayed scan (D–F) with opacification of both the
Fontan and more uniform opacification of the bidirectional Glenn allows assessment for obstruction and thrombi.
narrowing or as diffuse hypoplasia. It is generally progressive and has been stenosis (discrete versus diffuse hypoplasia) while also allowing precise
associated with neoproliferation of myofibroblast cells 68. measurements. Fig. 7A and B represent CCT imaging of infracardiac.
CCT is an excellent modality for evaluation of TAPVC or PAPVC as TAPVC in a newborn prior to surgical intervention. Fig. 8A and B
well as pulmonary vein stenosis. It is often used as a supplemental non- depicts a CCT performed in a 4-year-old child with a superior sinus
invasive imaging modality in conjunction with echocardiography to venosus atrial septal defect with PAPVC of the right upper and middle
plan for surgical or catheter-based interventions given the advantage of pulmonary veins.
better spatial resolution when compared to MRI.69,70 It provides a CCT is also used for post-procedure evaluation in these patients to
detailed assessment of the number and caliber of anomalous veins, the assess the adequacy of repair, stent position and patency as well as
exact location of drainage, and the relationship of the surrounding assessment for recurrent stenosis. Postoperative pulmonary venous
structures i.e. airway, pulmonary arteries and atrial appendages, in order obstruction or stenosis is one of the most common complications of
to plan for repair.71 CCT allows evaluation of features that impact anomalous pulmonary vein repair with an incidence of 5%–18%.72 Fig. 9
management such as the presence, degree and type of pulmonary vein demonstrates a 3D reconstruction obtained from a CCT showing
Fig. 12. 3 year old with transverse arch hypoplasia and coarctation of the aorta. (A). Oblique sagittal MIP demonstrating the severe discrete aortic coarctation with a
mildly hypoplastic distal transverse arch. The left subclavian artery is distally displaced. (B). 3D reconstruction demonstrating the site of severe coarctation with post-
stenotic dilation and collaterals seen supplying the descending aorta. (C) A 15 year old patient with coarctation of the aorta s/p stent placement.
476
recurrent pulmonary vein stenosis of the right and left sided pulmonary intervention. If there is inadequate systemic blood flow, such as in HLHS,
veins after sutureless repair in an infant with history of prematurity. One initial surgical repair will be the Norwood operation which involves
of the pitfalls of assessment of pulmonary veins by CCT is that it does not atrial septectomy, Damus-Kaye-Stansel anastomosis (connection be-
provide flow assessment, which can be helpful in PAPVC, when trying to tween the hypoplastic native aorta and the main pulmonary artery which
decide its hemodynamic significance by estimating shunt burden will become the neo-aorta), aortic arch reconstruction and establishing
(Qp:Qs).69,73 Despite some of its limitations, CCT is now routinely used pulmonary blood flow with a shunt or a RV to PA conduit (Sano). There
for evaluation of pulmonary veins and is likely to continue playing a are cases where pulmonary and systemic circulations are balanced and a
significant role in their assessment. neonatal repair is not needed. Most single ventricle patients will then
undergo the second stage palliation at around 3–6 months of age, which
12. Single ventricle consists of anastomosis of the SVC to the pulmonary artery (Glenn or
Hemi-Fontan). The third stage (Fontan) is typically performed at 2–4
There are various types of CHDs that are classified as single ventricle years of age and involves anastomosis of the IVC to the PA.
lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left These patients require advanced diagnostics to plan intervention and
heart syndrome (HLHS), unbalanced atrioventricular canals (AVC) and monitor residual hemodynamic abnormalities after Fontan completion.
double inlet left ventricle. Patients with single ventricle physiology The standard of care for interstage assessment of single ventricle CHD has
typically undergo a three stage surgical approach which leads to a Fontan been invasive cardiac catheterization, but more recently non-invasive
palliation, with the first stage varying depending on the exact anatomy evaluation has been proposed for those at low risk of needing inter-
and physiology. If there is inadequate pulmonary blood flow, a shunt vention.74–76 Cardiac MRI has been used with good results, but has not
from the aorta to the PA or a ductal stent will be the initial surgical been widely adopted due to the need for anesthesia and relatively long
Fig. 13. Cardiac computed tomography (CCT) in a 1 month old with double aortic arch and possible respiratory symptoms. (A,B) Axial and coronal images at the level
of the double aortic arch showing both the right and left arch measure normal in size. The trachea is seen (*) and does slightly narrow at the level of the double aortic
arch. (C) A 3-D reconstruction showing unobstructed right and left aortic arches. The right arch (R) is slightly superior to the left arch and slightly larger. (D) 3D
rendering of the double aortic arch and trachea demonstrating the mild narrowing of the trachea.
477
scan times.77 These fragile patients are highest risk for adverse event with population with high cumulative comprehensive diagnostic risk, dose
anesthesia.78 Additionally, as patients live to adulthood, many have optimization is essential due to the combination of high medical radia-
metallic implant that can affect MRI image quality.79 tion exposure and increased intrinsic risk of malignancy.81
Increasingly, CCT is being used for evaluation of single ventricle CHD
due to favorable comprehensive risk profile. CCT scans can be performed 13. Aortic arch anomalies
with no or limited anesthesia or moderate sedation in young patients too
young to cooperate, and are associated with relatively low radiation dose Congenital anomalies of the aortic arch are common indications for
in the current era. Non-sedated CCT can be performed in neonates CCT. The most common aortic arch anomalies to be evaluated by CCT
requiring evaluation of complex single ventricle anatomy prior to first include coarctation of the aorta, interrupted aortic arch and vascular
stage palliation, often required in the setting of heterotaxy syndromes. In rings.
carefully selected patients who are unlikely to require intervention by Coarctation of the aorta accounts for 5–7% of all CHD and has an
cardiac catheterization, CCT can be used at the first or second stage, prior incidence of 0.3–0.4 in 1000 live births.82 This diagnosis may present in
to Fontan completion at centers with appropriate technology and staff infancy or in older children/adults. Pre-operative evaluation in infancy is
(Fig. 10). CCT provides visualization of potential thoracic vasculature usually sufficient with echocardiogram. However, advanced imaging is
which may require intervention with excellent correlation to findings at often indicated in older children and adults to better plan surgical or
the time of cardiac catheterization.80 CCT provides exquisite visualiza- transcatheter repair (Fig. 12A and B). CCT is very useful in young chil-
tion of systemic venous anatomy, Sano or Blalock Taussig Thomas shunt dren who may need anesthesia for a cardiac MRI but can avoid this with a
anatomy and anastomosis to the pulmonary arteries, aortic anatomy CCT given that it is a much shorter study.
including coronary arteries and the proximal and distal Norwood Interrupted aortic arch is quite rare in comparison to coarctation of
anastomosis. the aorta, occurring in 0.7–1.4% of patients with CHD.83 This CHD is
In CHD patients after Fontan completion, CCT is an excellent imaging almost exclusively diagnosed in infancy although there are very rare
modality for evaluation of both ventricular function and Fontan anatomy cases reported in adults.84 Similar to coarctation of the aorta,
in select patients with MRI restrictions as noted. Contrast injection pro- pre-operative assessment is typically performed with echocardiogram. If
tocols must be adjusted to avoid misinterpretation of unpacified venous there are anatomic details which are not clearly delineated CT can be
streaming as Fontan thrombus on a routine CCT scan of the chest. A performed for a more detailed assessment.85
delayed scan with a high contrast dose is optimal to allow time for There is a postoperative risk of re-coarctation from coarctation or
contrast to fill the inferior limb of the Fontan pathway and allow clear interrupted aortic arch repair as well as a risk of aneurysm formation
visualization of the Fontan pathway (Fig. 11). A delayed scan is also near the site of repair.86 It is recommended that advanced imaging
optimal to evaluate for clot within a residual ventricular chamber or routinely be performed in patients status post repair at regular in-
within the Fontan pathway and pulmonary arteries. A test bolus can also tervals. This is most typically done with cardiac MRI. In patients with
be considered to optimize scan timing. Functional scans can also be used contraindications to MRI or those with aortic stents, CCT is often used
to evaluate for fenestration flow and for venous occlusions and collateral (Fig. 12C).33,87,88
vessels. 3D and four dimensional (3D þ motion) imaging can aid inter- Suspected vascular rings are optimally imaged by CCT, as it yields
ventional cardiologists and surgeons in planning for sternal entry and simultaneous visualization of the vascular structures as well as the
Fontan intervention. CCT may also be the modality of choice for patients airway. CCT is helpful in the diagnosis of vascular rings and can
needing assessment of coronary arteries in addition to congenital cardiac accurately assess for the presence of a diverticulum of Kommerell.89 It
anatomy. For patients with single ventricle CHD, and in many other areas aids in surgical planning including determining location of division of
of congenital cardiology, non-invasive assessment is increasingly used for the ring and if other procedures such as aortopexy or tracheal recon-
routine assessment of anatomy and function, allowing invasive cathe- struction are necessary (Fig. 13). Advanced imaging is rarely required
terization to be utilized primarily for intervention. postoperatively, although in cases of suspected continued airway nar-
Patients with single ventricle heart disease are increasingly living to rowing CCT may be helpful to best define any vascular compression of
adulthood, and cumulative radiation dose is of concern. For this patient the airway.90
Fig. 14. Post-processed cardiac computed tomography images of an anomalous aortic origin of the right coronary artery. (A) Multiplanar reformat windowed to show
the hypointensity of epicardial fat (*) and the lack of peri-coronary fat seen adjacent to the interarterial course of the anomalous coronary artery (arrow), suggestive of
an intramural course. Note the increase height:width ratio resulting in an elliptical lumen, also suggestive of an intramural course. (B) These signs are used on
multiplanar reformatting to measure an estimated intramural length. (C) 3D reconstruction confirms that the intramural course (yellow arrows) is above the STJ and
remote from the intercoronary commissural post (yellow *). Left sinus of Valsalva (L SoV) and right sinus of Valsalva (R SoV). (For interpretation of the references to
colour in this figure legend, the reader is referred to the Web version of this article.)
478
14. Anomalous aortic origin of the coronary artery terize the angle of take-off, proximal course of the AAOCA, identify if there
is an interarterial course and whether it is intramural vs. intraconal (or
Sudden cardiac death (SCD) is the leading medical cause of death in intramyocardial). Intramurality and intramural length can be determined
athletes, with coronary anomalies being one of the most common find- by the presence of an elliptical lumen (higher height:width ratio) and the
ings on autopsy in the young athlete population.91–93 Despite the amount absence of peri-coronary fat (Fig. 14A and B). Intramural length can be
of visibility and attention that SCD in an athlete receives, there is still estimated with good agreement to intraoperative findings.97 Other tech-
much that remains unknown regarding the risk factors and triggers for niques, such as virtual angioscopy and 3D reconstructions (Fig. 14C) can
SCD, especially in those diagnosed with an anomalous aortic origin of the help the imager and cardiothoracic surgeon characterize the lesion
coronary artery (AAOCA). Given the catastrophic, but potentially pre- (Fig. 15). Advanced digital modeling from CCT has also been utilized to
ventable, risks associated with an AAOCA, a comprehensive evaluation better delineate AAOCA characteristics and was found to best represent
with multimodality imaging is recommended.94,95 CCT plays a central ostial characteristics.98 Increasing height:width ratio has been correlated
role in the morphologic characterization and risk-stratification of the with increasing stenosis of the intramural segment.95 Additional features
lesion due to its high resolution, rapid acquisition, and accuracy in that are used to guide clinical decision-making include coronary dominance
comparison to intraoperative findings.96–98 and presence of additional abnormalities, such as myocardial bridging. Beta
A combination of post-processing techniques should be utilized to blockade and sublingual nitroglycerin can be utilized depending on the
adequately characterize the lesion. Multiplanar reformatting can charac- patients’ heart rate, to optimize coronary artery visualization.
Fig. 15. CT post-processed image compared to intraoperative images. (A) Intraoperative image of the anomalous RCA. The intramural length is measured by inserting
a probe into the coronary lumen and placing a suture at the point where the coronary becomes epicardial. (B) Virtual angioscopy demonstrating a large, round left
main coronary artery (LMCA) lumen, and a slit-like anomalous right coronary artery (RCA) lumen. The anomalous RCA is to the left of the intercoronary commissural
post (yellow *) and appears above the sinotubular junction (STJ). Similar in appearance to the intraoperative picture. (C) Post-operative image demonstrating
unroofing of the intramural segment and creation of a neo-ostium. (D) Cardiac computed tomography 3-months post-op with virtual angioscopy of the unroofed RCA.
Note the significantly increased size of the RCA lumen and similar “funnel-shaped” appearance seen on virtual angioscopy compared to the intraoperative imaging.
(For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
479
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Journal of Cardiovascular Computed Tomography 16 (2022) 467–482

Contents lists available at ScienceDirect

Journal of Cardiovascular Computed Tomography

State of the Art in Cardiovascular CT’

Cardiovascular computed tomography in pediatric congenital heart disease:

Abbreviations MRI magnetic resonance imaging

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