Cardiovascular MRI in Congenital Heart Disease
An Imaging Atlas
Shankar Sridharan · Gemma Price
Oliver Tann · Marina Hughes · Vivek Muthurangu
Andrew M. Taylor
Cardiovascular MRI
in Congenital Heart
Disease
An Imaging Atlas
13
From:
The Centre for Cardiovascular MR
UCL Institute of Child Health & Great Ormond Street Hospital for Children
Cardiovascular Unit
Great Ormond Street
London WC1N 3JH
UK
Dr. Shankar Sridharan
Locum Consultant Paediatric Cardiologist
Gemma Price
Medical Illustrator
Dr. Oliver Tann
Consultant In Cardiovascular Imaging
Dr. Marina Hughes
Consultant Paediatric Cardiologist
Clinical Lead for CMR
Dr. Vivek Muthurangu
BHF Intermediate Research Fellow
Professor Andrew M. Taylor
Professor of Cardiovascular Imaging
Director – Centre for Cardiovascular MR
ISBN 978-3-540-69836-4
eISBN 978-3-540-69837-1
DOI 10.1007/978-3-540-69837-1
Springer Heidelberg Dordrecht London New York
Library of Congress Control Number: 2009938029
© Springer-Verlag Berlin Heidelberg 2010
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V
Preface
The last 10 years has seen explosive expansion of the number of centres performing cardiovas-
cular magnetic resonance (CMR) imaging. The majority of this expansion has been in the field
of adult ischaemic imaging, but congenital heart disease remains one of the main indications
for CMR. Importantly, the greatly improved survival of patients with congenital heart disease
gives us a burgeoning adult population living with the sequelae of the disease (grown-up con-
genital heart disease – GUCH).
Without previous experience or formal training, the interpretation of CMR images of
patients with congenital heart disease can be difficult. The main aim of this book is to create a
portable resource that offers efficient access to high-quality MR (and where appropriate, CT)
images of the common congenital and structural heart abnormalities. We hope that by provid-
ing key images for each condition and a clear interpretation of the MR appearances, we will
improve the reader’s understanding of the conditions, facilitate their interpretation of images
and optimise the planning of the imaging protocols during their own practice of congenital
CMR.
As with any publication from a single institution, the contents of this book represent our
own practice. We have not written a definitive or exhaustive description of the conditions.
However, we hope that we have produced a factual, simple and eye-pleasing guide for fellows
training in CMR, radiographers and technicians performing CMR scans, physician users of
CMR, and perhaps those few in adult ischaemic practice, who may need the occasional aide
memoir for incidental findings!
We hope that you will find this book useful in your everyday practice and learning.
Shankar Sridharan
Gemma Price
Oliver Tann
Marina Hughes
Vivek Muthurangu
London, UK
Andrew Taylor
VII
Contents
1
2
3
4
5
6
7
8
Technical Considerations. . . . . . . . . . . . . . . . . . .
MR Imaging Under GA. . . . . . . . . . . . . . . . . . . .
Imaging Protocol . . . . . . . . . . . . . . . . . . . . . . . . .
Normal Anatomy-Axial. . . . . . . . . . . . . . . . . . . .
Normal Anatomy-Coronal. . . . . . . . . . . . . . . . . .
Normal Anatomy-Sagittal . . . . . . . . . . . . . . . . . .
Image Planes-Ventricles . . . . . . . . . . . . . . . . . . .
Imaging Planes-
Left Ventricular Outflow Tract . . . . . . . . . . . . . .
1
2
3
6
8
10
12
14
32 Tetralogy of Fallot: Repaired. . . . . . . . . . . . . . . . 78
33 Pulmonary Stenosis . . . . . . . . . . . . . . . . . . . . . . . 84
34 Percutaneous Pulmonary Valve
Implantation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86
35 Pulmonary Atresia and VSD . . . . . . . . . . . . . . . . 90
36 Transposition of the Great Arteries:
Arterial Switch Operation . . . . . . . . . . . . . . . . . . 92
37 Transposition of the Great Arteries:
Senning and Mustard Repair . . . . . . . . . . . . . . . . 96
9 Imaging Planes-
38 TGA with VSD and PS . . . . . . . . . . . . . . . . . . . . 100
Right Ventricular Outflow Tract . . . . . . . . . . . . .
10a Imaging Planes-Branch PAs . . . . . . . . . . . . . . . .
10b Imaging Planes-Thoracic Aorta. . . . . . . . . . . . . .
11a Imaging Planes-Tricuspid Value . . . . . . . . . . . . .
11b Imaging Planes-Mitral Value . . . . . . . . . . . . . . . .
12 Imaging Planes-Coronary Arteries . . . . . . . . . . .
13 Atrial Septal Defect . . . . . . . . . . . . . . . . . . . . . . .
14 Sinus Venosus Defect . . . . . . . . . . . . . . . . . . . . .
15 Atrioventricular Septal Defect. . . . . . . . . . . . . . .
16 Ventricular Septal Defect. . . . . . . . . . . . . . . . . . .
17 Aortic Valve Stenosis. . . . . . . . . . . . . . . . . . . . . .
18 Aortic Valve Incompetence . . . . . . . . . . . . . . . . .
19 Coarctation of the Aorta . . . . . . . . . . . . . . . . . . .
20 Repaired Coarctation of the Aorta:
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . .
21 Interrupted Aortic Arch . . . . . . . . . . . . . . . . . . . .
22 Aortic Vascular Rings . . . . . . . . . . . . . . . . . . . . .
23 Left Pulmonary Artery Sling . . . . . . . . . . . . . . . .
24 Marfan Syndrome . . . . . . . . . . . . . . . . . . . . . . . .
25 Williams Syndrome . . . . . . . . . . . . . . . . . . . . . . .
26 Mitral Stenosis. . . . . . . . . . . . . . . . . . . . . . . . . . .
27 Mitral Regurgitation . . . . . . . . . . . . . . . . . . . . . .
28 Hypertrophic Cardiomyopathy . . . . . . . . . . . . . .
29 Dilated Cardiomypathy . . . . . . . . . . . . . . . . . . . .
30 Noncompaction Cardiomyopathy . . . . . . . . . . . .
31 Tetralogy of Fallot . . . . . . . . . . . . . . . . . . . . . . . .
16
18
19
20
21
22
24
26
28
32
36
40
42
46
48
50
54
56
58
62
64
66
70
72
74
39 Congenitally Corrected Transposition
of the Great Arteries . . . . . . . . . . . . . . . . . . . . . . 104
40 Common Arterial Trunk . . . . . . . . . . . . . . . . . . . 108
41 Double Outlet Right Ventricle . . . . . . . . . . . . . . . 112
42 Double Inlet Left Ventricle . . . . . . . . . . . . . . . . . 116
43 Hypoplastic Left Heart Syndrome:
Norwood Stage 1 . . . . . . . . . . . . . . . . . . . . . . . . . 118
44 Bi-directional Cavo-pulmonary
(Glenn) shunt . . . . . . . . . . . . . . . . . . . . . . . . . . . . 122
45 Fontan-Type Circulation
(Tricuspid Atresia). . . . . . . . . . . . . . . . . . . . . . . . 126
46 Total Cavo-pulmonary Connection . . . . . . . . . . . 130
47 Anomalous Coronary Arteries. . . . . . . . . . . . . . . 134
48 Anomalous Left Coronary Artery
from Pulmonary Artery . . . . . . . . . . . . . . . . . . . . 138
49 Kawasaki Disease . . . . . . . . . . . . . . . . . . . . . . . . 140
50 Total Anomalous Pulmonary
Venous Drainage . . . . . . . . . . . . . . . . . . . . . . . . . 144
51 Partial Anomalous Pulmonary
Venous Drainage . . . . . . . . . . . . . . . . . . . . . . . . . 146
52 Ebstein’s Anomaly . . . . . . . . . . . . . . . . . . . . . . . 150
53 Right Isomerism . . . . . . . . . . . . . . . . . . . . . . . . . 154
54 Left Isomerism. . . . . . . . . . . . . . . . . . . . . . . . . . . 158
55 List of Abbreviations . . . . . . . . . . . . . . . . . . . . . . 162
56 Further reading . . . . . . . . . . . . . . . . . . . . . . . . . . 164
1
1
Technical Considerations
Paediatric Challenges
¼ The usual technical difficulties faced when perform-
ing a cardiac MR examination are further amplified
when imaging small children
¼ Optimal image quality may be compromised
because of
– The smaller size of structures
– Faster heart rates
– The reduced time for image acquisition (inability
or difficulty with breath-holding)
¼ The imaging protocol should be prioritised to obtain
the most crucial diagnostic information, in case the
patient’s cooperation is limited
1. Spatial Resolution
Smaller field-of-views (FOVs) and the use of thinner
slices are required to image small anatomical structures.
This leads to increased image resolution, but a
corresponding reduction in signal:noise (S/N) ratio.
This can be compensated by
¼ Increasing the number of acquisitions – (disadvan-
tage: increase in scan time)
¼ Removing parallel imaging features – (disadvan-
tage: increase in scan time)
¼ Using a coarser matrix, to increase diagnostic image
quality, albeit at the cost of reduced resolution
2. Appropriate Coil Selection
Appropriate coil selection is important to maximise
S/N ratio.
¼ A dedicated extremity (knee coil) should be used in
neonates or very small children.
¼ A transmit/receive coil can reduce noise and increase
S/N ratio.
¼ If the child is too large for this, then a body matrix
and spine coil combination achieves good results.
3. Faster Heart Rates
Faster heart rates in small children result in a short R-R
period.
¼ For sequences where repetition times (TR) are
longer than the R-R period, gating, using the sec-
ond or third R wave as the trigger facilitates more
time for the appropriate recovery of longitudinal
magnetisation.
¼ For cine-imaging, reducing the number of phase
encode steps in each frame will decrease the acqui-
sition period for each frame, improving temporal
resolution and image sharpness. However, this
increases scan times.
4. Strategies to Reduce Motion Artefact
¼ Play therapy or pre-examination visits to the scan-
ner can help a child overcome any anxiety and
improve in-magnet stillness.
¼ Installing a DVD/Video system is a worthwhile
investment to promote prolonged distraction and
cooperation.
¼ For children who have difficulty breath-holding,
images can be acquired during free breathing.
Additionally:
– Use manual shimming techniques, as they are
essential to minimise flow artefacts, particularly
on balanced SSFP sequences
– Increase the number of acquisitions (NEX) from
1 to 3
– Use respiratory compensation methods to
acquire data, e.g. use of navigator echoes, phase
re-ordering algorithms.
– Acquire data using real time imaging sequences
(where imaging systems allow).
5. Contrast Administration in Children
For angiography, we use 0.2–0.4 mL/kg of Dotarem,
(Guerbet, Paris) which corresponds to 0.1–0.2 mmol/
kg. All Gadolinium contrast agents need to be given
in accordance with Institutional and National guide-
lines to avoid nephrogenic sytemic fibrosis (NSF).
For further information on this, see the UK Royal
College of Radiologists document on this subject:
h t t p : / / w w w. r c r. a c . u k / d o c s / r a d i o l o g y / p d f /
BFCR0714_Gadolinium_NSF_guidanceNov07.pdf
6. Consider Alternative Imaging Strategies
CT is potentially useful if MR assessment is limited or
hampered by technical restraints.
2
Cardiovascular MRI in Congenital Heart Disease
2
MR Imaging Under GA
Indications for General Anaesthesia (GA)
for Paediatric MR
Practice varies throughout the world. However, most
centres in the UK will perform cardiovascular MR
under general anaesthetic (GA) for children under the
age of 7 years.
General Safety Issues Specific to Paediatric
Cardiac Imaging
¼ Patient metal checked and the safety questionnaire
performed with parents before the child is
anaesthetised.
¼ Senior cardiac anaesthetist continuously present in
every case.
¼ Full monitoring: pulse oximetry, end-tidal gas anal-
ysis, ECG and non-invasive BP.
¼ Wrap the patient in gamgee or blankets to keep him
or her warm.
¼ Ten metre circle breathing system needed, to link
the patient to anaesthetist in MR control room.
¼ Breath-holding in passive expiration, controlled by
Technical Factors Specific to MR in Infants
and Small Children
¼ Prolonged, multiple breath holds are required. This
can cause hypoxia. Adequate pause for ventilation
control between breath holds is required.
¼ A reliable ECG is vital for gating during image
acquisition.
¼ Monitor patient temperature closely. The low ambi-
ent temperature in MR scanning room produces a
hypothermia risk, particularly for small infants.
breaking the circuit in the control room.
¼ The large dead space prohibits low flow
anaesthesia.
¼ Reversal of anaesthesia and extubation in CMR
induction room. Ensure that the team is aware of the
cardiac arrest procedure.
Importantly, the child MUST be withdrawn from the
MR room for resuscitation. Metallic objects such as
resuscitation trolley MUST NOT be brought into the
scanning room.
Environmental and Physical Constraints
Performing general anaesthesia (GA) in a magnetic
resonance (MR) environment is challenging for many
reasons
¼ During the scan, there is limited access to the child
Power
injector
Anaesthetic
machine
Physiological
monitoring
and ventilation equipment.
¼ Care is required for staff and patient safety with
regard to ferromagnetic equipment.
¼ There is a potential for RF interference with moni-
toring equipment.
Fig. 2.1. Photography showing one of our dedicated
paediatric cardiac MR labs. Inset, control room with
monitoring equipment and long anaesthetic tubing to
enable the anaesthetist to sit in the controlroom during
MR scanning
3
Imaging Protocol
3
Imaging Protocol
3
4
Cardiovascular MRI in Congenital Heart Disease
3
Imaging Protocol
5
6
Cardiovascular MRI in Congenital Heart Disease
4
Normal Anatomy-Axial
The transverse or axial plane is useful for studying morphology and the relationships of the
four cardiac chambers and the pericardium.
Images (a) through (f ) show axial planes in a head to foot direction.
a
f
InA
a
BCV
LCC
LSA
SVC
Azy
b
AAo
DAo
4
Normal Anatomy-Axial
7
SVC
AAo
RAA
LAA
PT
AAo
RPA
LPA
LA
c
DAo
d
DAo
RVOT
e
RAA
RLPV
Ao
LA
f
RA
IVC
RV
DAo
LV
8
Cardiovascular MRI in Congenital Heart Disease
5
Normal Anatomy-Coronal
Frontal or coronal images are most useful for investigation of the LVOT, of the left atrium,
and the pulmonary veins.
Images (a) through (f ) show coronal planes through the heart in an apex-to-base direction.
f
a
a
c
SVC
RA
Ao
RV
PT
LV
LV
b
RCA
d
RV
RPA
LV
RVOT
Ao Arch
LPA
5
Normal Anatomy-Coronal
9
RV
IVC
RA
LV
e
Ao Arch
LPA
f
LPA
LA
RLPV
LAA
LLPV
DAo
10
Cardiovascular MRI in Congenital Heart Disease
6
Normal Anatomy-Sagittal
Saggital images can be used to study the connections between the ventricles and the great
vessels.
Images (a) through (f ) show saggital planes through the heart in a right-to-left direction.
a
f
a
SVC
c
RA
IVC
b
SVC
RV
d
RA
Azy
6
Normal Anatomy-Sagittal
11
AAo
DAo
RPA
LA
RVOT
LA
RV
RV
e
PT
RV
LV
DAo
f
LV
LV
12
Cardiovascular MRI in Congenital Heart Disease
7
Image Planes-Ventricles
Planned using HASTE axial stack, and acquired using a b-SSFP sequence.
These figures illustrate the process with normal anatomy. The same principles are used for
patients with any ventricular arrangement.
a
b
Fig. 7.1. The LV vertical long axis plane (LVLA) is aligned using the axial plane, through the
mitral valve and the LV apex, which may be on a separate more inferior slice
a
b
Fig. 7.2. The RV vertical long axis plane (RVLA) is aligned using the axial plane, through the
tricuspid valve and the RV anterior wall – apex
7
Image Planes-Ventricles
13
a
b
c
Fig. 7.3. The short axis plane (SA) is aligned using the VLA views and the axial plane, and is perpendicular to both
a
c
b
d
Fig. 7.4. The 4-chamber view is planned using the SA and the VLA views. A perpendicular
line is placed through the anterior mitral valve papillary muscle and the apex of the RV in the
SA, and then adjusted to intersect the LV and RV apices in VLA views
14
Cardiovascular MRI in Congenital Heart Disease
8
Imaging Planes-Left Ventricular Outflow Tract
Planned and acquired using b-SSFP sequence.
LVOT view useful for septal hypertrophy and LVOT obstruction in HCM.
Aortic valve plane used for through-plane Ao flow, and valve morphology.
These figures illustrate the process with normal anatomy. The same principles are used for
patients with any ventricular arrangement.
a
c
b
d
8
Imaging Planes-Left Ventricular Outflow Tract
15
Fig. 8.1. Imaging planes can be aligned from the basal SA slice (a). The LV inflow (mitral valve)/outflow (aortic
valve) view (b) acquired with a plane passing across mid-point Ao and mitral valves on the basal SA slice. A per-
pendicular cross-cut of the LVOT produces image (c). Alignment of the aortic valve plane (d) for aortic flow assess-
ment from the LV inflow/ outflow (b) and the left ventricular outflow tract (LVOT) (c) views (dotted lines). The
imaging plane should be placed just above the aortic valve, yet just below the origin of the coronary artery origins
16
Cardiovascular MRI in Congenital Heart Disease
a
9
Imaging Planes-Right Ventricular
Outflow Tract
RVOT planned using HASTE axial images, and acquired using b-SSFP.
Pulmonary valve plane used for through-plane PA flow, and valve morphology.
These figures illustrate the process with normal anatomy. The same principles are used for
patients with any ventricular arrangement.
b
c
e
Pulmonary outflow
d
9
Imaging Planes-Right Ventricular Outflow Tract
17
Fig. 9.1. Alignment of the pulmonary valve plane (e)
for pulmonary flow assessment from two perpendic-
ular right ventricular outflow tract (RVOT) (c, d)
views (dotted lines). The imaging plane should be
placed just above the pulmonary valve. The first
RVOT view is prescribed from an oblique plane
through the main pulmonary artery and RV on a set
of axial images (a, b). The second RVOT plane is
prescribed perpendicular to the first RVOT view
(complete line on (c))
18
Cardiovascular MRI in Congenital Heart Disease
a
c
10a
Imaging Planes-Branch PAs
The branch PAs (RPA and LPA) do not lie in the same axial plane (LPA is slightly superior to
the RPA).
b
d
Fig. 10.1 (a-d). Both arteries can be shown in an oblique axial plane (a). An oblique coronal plane can be used
to show the RPA (b). An oblique sagittal plane used to show the LPA (c). PA MRA shown in (d).
Imaging Planes-Thoracic Aorta
19
10b
Imaging Planes-Thoracic Aorta
The thoracic aorta has a complex morphology, which does not lie in a single saggital or
coronal plane.
e
h
f
i
g
Fig. 10.1 (e -i). Alignment of the thoracic aorta using a 3-point plan. Points placed on “black-blood” axial
images: (e) ascending aorta, (f) aortic arch, and (g) proximal descending aorta. The in-plane image of the tho-
racic aorta in (h) shows a tight