Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI

Full text

ISSN:2155-9880
The International Open Access
Journal of Clinical & Experimental Cardiology
Special Issue Title:
Congenital Heart Disease-Recent Discoveries and
Innovations
Handling Editors
Georg Hansmann
Children’s Hospital Boston, USA
Matthias Sigler
Georg-August University Goettingen, Germany
This article was originally published in a journal by OMICS
Publishing Group, and the attached copy is provided by OMICS
Publishing Group for the author’s benet and for the benet of
the author’s institution, for commercial/research/educational use
including without limitation use in instruction at your institution,
sending it to specic colleagues that you know, and providing a copy
to your institution’s administrator.
All other uses, reproduction and distribution, including without
limitation commercial reprints, selling or licensing copies or access,
or posting on open internet sites, your personal or institution’s
website or repository, are requested to cite properly.
Available online at: OMICS Publishing Group (www.omicsonline.org)
Digital Object Identier: http://dx.doi.org/10.4172/2155-9880.S8-008

Review Article Open Access
Clinical & Experimental
Cardiology
Steinmetz et al., J Clinic Experiment Cardiol 2012, S:8
http://dx.doi.org/10.4172/2155-9880.S8-008
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
*Corresponding author: Michael Steinmetz, MD, Department of Pediatric
Cardiology and Intensive Care Medicine, University Hospital Gottingen, Robert-
Koch-Str. 40, 37099 Gottingen, Germany, Tel: ++49-551-3922550; Fax: ++49-551-
3922560; E-mail: michael.steinmetz@med.uni-goettingen.de
Received January 26, 2012; Accepted March 04, 2012; Published March 07,
2012
Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for
Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic Experiment
Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Copyright: © 2012 Steinmetz M, et al. This is an open-access article distributed
under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited.
Abstract
Cardiac magnetic resonance imaging (CMR) has become an important tool in evaluating congenital heart
disease (CHD) in children and adults. By learning more about the advantages and limitations of CMR, clinicians
and surgeons increasingly use the images and data acquired by CMR for the management of patients with CHD.
MRI technology is evolving fast, and techniques such as 3D-MR angiography, phase contrast ow measurements,
functional images to quantify cardiac function and stress testing are nowadays integrated parts of clinical care for
patients with CHD. New technologies involve 4D-Flow measurement, “real-time” MRI or as a more future perspective
MRI based catheter interventions.
This article intends to give an overview over the role of CMR in CHD, with a special focus on the latest
development of the past 5 years and an outlook to the techniques on the horizon.
Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in
Cardiac MRI
Michael Steinmetz1*, Hendrik C Preus1 and Joachim Lotz2
1Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital Gottingen, Germany
2Department of Diagnostic Radiology, University Hospital Gottingen, Germany
Abbreviations: 3D/ 4D: ree/ Four Dimensional; AAO:
Ascending Aorta; APA: Atrio Pulmonary Anastomosis; APVR:
Anomalous Pulmonary Venous Return; ARVC: Arrhythmogenic Right
Ventricular Cardiomyopathy; ASO: Arterial Switch Operation/ Jatene
Procedure; AV: Atrio-Ventricular; ccTGA: Congenitally Corrected
TGA; CHD: Congenital Heart Disease; CMR: Cardiac Magnetic
Resonance Imaging; CoA: Coarctation of the Aorta; CT: Computed
Tomography; DAO: Descending Aorta; ECG: Electro Cardiogramm;
EDV: End-Diastolic Volume; EF: Ejection Fraction; ESV: End-
Systolic Volume; ET: Extracardiac Tunnel; GRE: Gradient-Echo; HO:
Homogra; HOCM: Hypertrophic Obstructive Cardiomyopathy;
ICD: Implanted Cardioverter Debrillator; IVC: Inferior Caval Vein;
LA: Le Atrium; LGE: Late Gadolinium Enhancement; LIT: Lateral
Intracradiac Tunnel; LV: Le Ventricle; MAPSE: Mitral Annular Plane
Systolic Excursion; MRI: Magnetic Resonance Imaging; NCC: Non-
Compaction Cardiomyopathy; PA: Pulmonary Artery; PDA: Patent
Ductus Arteriosus; PR: Pulmonary Regurgitation; PV: Pulmonary
Valve; PVC: Premature Ventricular Contraction; Qp: Pulmonary
Blood Flow; Qs: Systemic Blood Flow; RA: Right Atrium; RV: Right
Ventricle; RVOT: Right Ventricular Outow Tract; SSFP: Steady-State
Free Precession; SVC: Superior Caval Vein; TAPSE: Tricuspid Annular
Plane Systolic Excursion; TCPC: Total Cavo Pulmonary Connection;
TGA: Transposition of the Great Arteries; TOF: Tetralogy of Fallot;
VR: Virtual Reality; VSD: Ventricular Septal Defect
Introduction
Patients with congenital heart disease (CHD) are a challenge for
imagers, since CHD requires a profound knowledge of the morphologic
and functional characteristics of a broad range of congenital heart
defects. Moreover, complex congenital heart disease oen involves
complex palliative or corrective surgery that alters the “normal” heart
anatomy and cardiac function profoundly [1,2]. e number of patients
reaching adulthood aer correction or palliation of complex congenital
heart disease is increasing signicantly, thereby creating a whole new
group of patients with complex chronic cardiac disorders [3,4].
Traditionally, imaging of CHD has been a domain of cardiac
catheterizations and echocardiography. e last ten years have seen the
rise of MRI and CT as accepted imaging modalities for congenital heart
disease. Especially MRI has been the source of important insights into
individualized pathophysiologic changes in CHD for both morphologic
and functional aspects [5,6]. However, no single imaging modality
has been shown to be able to obtain all information necessary for the
complete evaluation of patients with CHD. Echocardiography is non
invasive, possesses a high spatial and temporal resolution, but is oen
limited in its usability due to poor acoustic windows [3]. Assessment
of valvular morphology and function is unmatched by any imaging
modality in its temporal and spatial resolution [7].
In the cath lab, contrast agent volume and catheter position
inuence the degree of regurgitation, shunt magnitude, thereby
introducing a bias and decreased stability in the assessment of cardiac
functional parameters. However, invasive catheterization still remains
the only reliable way for pressure mapping of the cardiac chambers and
connected blood vessels as well as the assessment of coronary arteries.
Both modalities - echocardiography as well as invasive
catheterization – have limitations in the assessment of complex
anatomic alterations of the cardiac chambers and connected vessels.
One of the main reasons for the success of MRI in the diagnosis and
follow up of CHD is its ability to deliver detailed 3D imaging of the
complex anatomy before as well as aer surgical interventions [8].
Especially CMR has demonstrated added value of diagnostic
accuracy in the functional assessment of the right ventricle as well as in
its ability of imaging function and morphology in any spatial plane [9].
Minimal doses of contrast agents if any are needed for 3D angiography
in CMR. In the recent years some concerns have been issued about
the safety of MR contrast agents due to reports about nephrogenic

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 2 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
brosis caused by gadolinium based contrast agents. However, cyclic
gadolinium based MR contrast agents are now considered safe and
non-nephrotoxic in the young and grown-up patients. e lack of
radiation exposure is an important advantage of MRI in comparison to
multislice-CT or uoroscopy.
Spatial resolution of CMR is inferior to multislice-CT or
echocardiography. Most of the CMR-techniques employed today need
multiple heart cycles to obtain all data needed. As a result functional
data displayed are interpolations of multiple heart beats and not real-
time acquisitions as compared to echocardiography. erefore CMR
imaging in the newborn and young children usually need intubation
narcosis for high-resolution imaging. ere are quite a number of sites
that prefer deep sedation of the children up to the age of six limiting
CMR exams to morphologic questions and accepting rough estimates
for cardiac function. New developments allow for the synchronization
of MR imaging with both breathing and cardiac motion.
MRI is quite sensitive to local disturbances of the magnetic
eld. Especially stainless steel and materials alike do induce severe
disturbances of the local magnetic eld yielding artifacts in the images
that usually are much larger than the object causing them. ese
susceptibility artifacts are minimal with non-ferromagnetic materials
like Nitinol. Stainless steel clips, valves, valve prostheses, stentgras
all can cause susceptibility artifacts that can render an MRI exam
nondiagnostic.
Techniques/ Imaging Sequences Available in CMR for
Evaluation of CHD
Spin echo imaging with dark blood preparation
Anatomic imaging with high spatial resolution and suppressed
blood signal. It helps to show anatomical relations of cardiac and
extracardiac structures. In its fast variant, this sequence type can be
used to image the whole chest in one to two breath holds with some
compromises in image quality. Same sequence class can be used to look
for myocardial edema.
Cine imaging
By ECG-triggered gradient-echo (GRE) and steady-state free
precession (SSFP) sequences, a cardiac cycle can be resolved into
multiple phases. ese cine loops are recorded in dened planes and
allow for quantication of cardiac function, mass and ventricular
volumes. Moreover, qualitative assessment of wall motion, valve
function, and identication of intra-cardiac or inter-vascular shunts is
possible. Cine GRE measurements are more robust in terms of image
quality and are better suited for the visualization of ow jets resulting
from stenosis or insuciencies. SSFP sequences provide a superb
and homogenous contrast between blood pool and myocardium.
SSFP sequences therefore are generally preferred for the visual and
quantitative analysis of wall motion and cardiac function in general.
Velocity encoded phase contrast MRI for ow quantication
In- or through-plane measurements of ow velocity and thus
quantication of cardiac output, stroke volume and calculation of
shunt volume is possible [6,10]. To localize a high velocity intracardiac
shunts or in the search of the maximum velocity, in-plane ow maps
can be helpful.
3D- angiography with gadolinium enhancement
is technique is an excellent tool to show arterial and venous
structures, shunt connections or anomalous vascular morphology
or connections like aortic ectasia, coarction of the aorta, anomalous
pulmonary venous drainage, focal or diuse pulmonary artery stenosis,
collaterals. It is also very helpful for 3D-visualization of the vascular
anatomy to surgeons in preparation of complex surgical procedures
and to exemplify the relationship to other cardiac and extracardiac
structures [11].
Perfusion and dobutamine/ adenosine stress testing
Evaluation of myocardial ischemia due to coronary stenosis can
be detected indirectly by perfusion imaging or wall motion analysis
under pharmacologic stimulation. Perfusion imaging uses adenosine
(140 µg/kg Bodyweight x min) to induce maximal dilation of the
coronary arteries. During the dilation a short bolus of contrast agent is
injected intravenously and the passage of the contrast agents through
the myocardium is documented in a near-real time imaging. Coronary
artery stenosis cause the dependent myocardium to remain darker than
myocardium dependent on healthy coronaries as less contrast media
nds its way beyond the stenosis.
Dobutamine stimulation is used in combination with wall motion
analysis. Dobutamine is infused at increasing rate (5 to 40 µg / kg
Bodyweight, steps of 10 µg every 5min). During every dobutamine
level, wall motion is documented in MRI in at least 4 planes. Any new
wall motion analysis under infusion of dobutamine is regarded as an
evidence of a stenosis of the coronary artery supplying this myocardial
wall segment.
Late gadolinium enhancement (LGE)
Late Gadolinuim Enhancement (LGE) is an integral part of imaging
of cardiovascular disease and is found in a variety of cardiac diseases
[12]. It helps to detect diseased myocardium from scars, brosis,
deposition of material in extracellular space like amyloid, glycosides
or alike. In congenital heart disease, LGE has been successfully used
as a predictive indicator for ventricular function in patients aer
repair of TOF [13], TGA aer arterial switch [14] or aer Fontan
palliation [15]. It has been found to be associated with increased risk
of arrhythmias and sudden cardiac death in patients with hypertrophic
cardiomyopathy [16] or coronary artery disease due to atherosclerosis
[17] or surgical reimplantation [18]. Technically, late enhancement is
based on a T1 weighted MR sequence with a special pulse to render
normal myocardium black. e imaging sequence is done 12 to 15
min. aer the injection of Gadolinium based contrast agent. Normal
myocardium remains dark whereas diseased myocardium takes up
various amounts of signal due to retained contrast material.
Early gadolinium enhancement
In comparison to Late Enhancement, the idea of Early Enhancement
indicates a myocardial hyperemia or hyperperfusion as can be found
in active myocarditis of any cause. Early Gadolinium Enhancement
therefore is done immediately aer the injection of intravenous
contrast. As it might be dicult to visualize the subtle enhancement
of hyperperfused myocardium, signal intensities of the myocardium
are oen compared to that of the pectoral muscles or paravertebral
muscles.

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 3 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
Future Outlook/ Techniques on the Verge of Clinical
Application
3D-/ 4D-Flow
Newer methods allow ow measurements of 3D-volumes thereby
providing spatial and temporal resolutions of complex ow situations
[19,20]. Data can be processed aer the scan and ow or velocities
can be measured at dierent locations within the data set. Especially
palliated single ventricle anatomies with Fontan circulation have been
looked at by 4D-Flow quantication [20]. Here, vortex formation and
ow in the Fontan circulation may help determine prognosis and
timing for intervention, surgery or heart transplantation [21]. However,
further studies are needed to evaluate the use of this technique in
clinical routine. Temporal resolution is inferior to that of 2D ow
measurements and though quite some advances have been made, time
required for a 3D ow measurement remains at 5 to 10 minutes.
Real time MRI
One of the major drawbacks of MR imaging is the lack of high-
resolution real time image. It would allow MRI examinations not
aected by arrhythmias and also enabling free breathing whilst
obtaining high-resolution MR images of function or morphology.
Several approaches have been proposed to the goal of real-time imaging.
Echoplanar imaging has been employed but the images are susceptible
to devastating artefacts and have inferior spatial resolution. Other
approaches use highly accelerated imaging techniques like massive
parallel imaging techniques in combination with sampling only a
minute amount of data normally necessary for traditional MR imaging.
ough insular solutions have been reported, real-time imaging still is
in the laboratory phase and only available at specic research sites. It
has been reported to help in the dierentiation of constrictive versus
restrictive myocardial disease by evaluation of the ventricular septal
movement during valsalva manoeuvre.
MR based catheterization laboratory
Advances are being made in order to bring the advantages of
invasive cathertization under uoroscopy – interventions and invasive
hemodynamics - to the CMR [22]. Various animal studies have
demonstrated the feasibility of MR based catheterization techniques
such as coronary intubation [23], balloon valvuloplasty of the aortic
valve [24], fusion of x-ray and CMR-roadmaps for device based VSD-
closure [25], intravascular angioplasty and stent placement [26-29] or
assessment of ventricular function [30]. New CMR methods analogous
to selective catheter-angiography to visualize ow using virtual dye
are being developed [31]. Patient safety is the major concern in the
development of interventional instrumentation. e development of
CMR suitable catheters, stents, balloons and MR sequences is under
way, but still not available for routine use in humans [22,31-33].
However, some investigators see the practical implementation of the
MRI based catheterization lab on the horizon [34,35]. One important
step to implement MRI based angiographies similar to those known
from the conventional catheter lab using uoroscopy would be a robust
spatial as well as temporal real time MRI technique.
Whole heart sequences, 3 D reconstruction and 3 D hardprints
for operation planning/ 3D virtual surgery
Virtual reality 3D-reconstruction is a standard tool in CMR.
Newer techniques apply this for more detailed planning of surgery
using rasin-based hardprints of the 3D-models so that a surgeon can
physically put his hands on a and look at the complex anatomy from
all sides according to the individual needs [36]. To take this even
further, 3D-sequences are used to create models for virtual surgery,
so that the surgeon may simulate his operation in a computed model
before performing the surgery on the actual patient [37,38]. Although
these are interesting and helpful techniques for a more individualized
treatment, further steps to implement them in routine clinical practice
will have to be done.
Special Congenital Malformations
Tetralogy of fallot (TOF )
Tetralogy of Fallot (TOF) is the most common cyanotic cardiac
defect accounting for approx. 10% of all CHD. TOF patients have
been examined by CMR more than any other group of patients with
CHD. RV function and size, regional dysfunction, scarring, degree of
pulmonary regurgitation (PR) and pulmonary arterial anatomy can be
assessed reliably (Figure 1). ese data are used increasingly for risk
stratication and timing of repetitive valve replacements, interventions
for peripheral pulmonary stenosis or antiarrhythmic therapy with
drugs or pacemakers/ ICDs. Moreover, CMR is very useful to plan
operative or interventional steps in the follow-up aer TOF repair. 3D
MR-angiography complements echo and catheterization techniques.
CMR provides exact three dimensional virtual models of the RV,
RVOT and the pulmonary arteries. In addition CMR in TOF also yields
a wealth of functional parameters that have become essential in clinical
decision making.
Oosterhof et al. have reported that RV EDV more than 160 mL/m2
or RV ESV more than 82 mL/m2 in CMR prior to repair of signicant
pulmonary regurgitation is associated with decreased chances of
normalization of RV volume [39]. RV-Dilation >150-160 ml/m2 with
decreased RV function is now regarded an important cut-o parameter
for the timing of pulmonary valve (PV) replacement. Gender specic
normal CMR values for ventricular volumes and myocardial mass for
children and adolescents have been published recently [40] as well
as for patients aer repair of TOF [41]. Gender specic percentiles
of CMR parameters in patients with repaired TOF computed for an
age range from 8 to 40 years show changes over time in LV volumes
especially in females and RV volumes in both male and females. Also bi-
ventricular ejection fraction (EF) decreased in male patients, whereas
in female patients only RV EF decreased. is should be considered
when dening thresholds for intervention. Gender-specic percentiles
for the individual patient may help in nding the optimal time point
for PV replacement [41].
e confounding inuence of residual right ventricular outow
tract (RVOT) obstruction combined with pulmonary regurgitation
(PR) vs. isolated PR aer TOF-repair has also been addressed lately.
Residual RVOT obstruction was associated with smaller RV-volumes
and higher RV-EF. us, TOF patients with residual RVOT obstruction
may need, earlier PV replacement, i.e. even when RV-volume is lower
than 160ml/m2 in CMR, compared to those with no RVOT obstruction
[42].
Another predictor for RV function and increased risk of sudden
cardiac death due to ventricular arrhythmia is the extent of ventricular
brosis. is can be assessed by late gadolinium enhancement in CMR
[13,15,43]. e more brosis detected in CMR by LGE, the higher the
risk for sudden cardiac death and deteriorating RV function.
Intra-(RV) and inter-ventricular, as well as atrio-ventricular
dependence has been addressed in recent CMR studies in repaired

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 4 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
TOF. Echocardiography parameters such as tricuspid- and mitral
annular plane systolic excursion (TAPSE, MAPSE), speckle tracking
and myocardial strain have been related to CMR ndings. eir
potential in routine clinical echo examination and their role as
predictive parameters for the timing of necessary interventions has
been evaluated [44-46]: A) e individual components of the RV react
dierently to volume overload [47]. B) Moderate systolic and diastolic
right ventricular dysfunction appears to be associated with impaired
right atrial function in TOF patients, which corresponds to decreased
TAPSE in echocardiography [45]. C) Despite normal EF on CMR
and echo, TOF patients exhibit a decreased 2D-longitudinal strain,
suggesting subclinical functional impairment [44].
e accuracy of 3D-echocardiography has also been compared to
CMR in TOF patients and has been shown to decrease signicantly
with increasing RV-sizes, while CMR values remain relatively robust
[48].
CMR is also used to plan percutaneous pulmonary valve
interventional (PPVI) replacement and document ventricular
improvement in follow up examinations [49]. Of note, MRI based
evaluation of pulmonary valve function can be limited aer stent
mounted interventional valve replacement, due to the artifacts
produced by metal stents.
Coarctation of the aorta (CoA)
e congenital narrowing of the aorta is frequently located in
close vicinity of the site of ductal insertion (aortic isthmus) [50]. In
infancy and with critical coarctation the treatment of choice is surgery.
Elder patients with less severe CoA and subjects with re-coarctation
aer initial surgical repair are eligible for interventional therapy using
balloon angioplasty or stent implantation [51-53]. Follow up of patients
aer corrective surgery for CoA can be dicult if the patient reaches
adult age. Echocardiography is very limited, once the patient is grown-
up, and the aortic arch is only poorly visualized.
Recent studies show that a considerable number (up to 50
percent) of patients aer corrective surgery for CoA develop aortic
abnormalities. However, a signicant number of these patients are
asymptomatic [54]. A common complication aer surgical repair of
coarctation is the development of re-coarctation or aortic aneurysms.
Patients with bicuspid aortic valves appear to be at a higher risk for
developing re-coarctation whereas patients aer surgical repair with
patch plasty rather tend to develop aortic aneurysms [55-57]. It is
therefore desirable to detect these abnormalities at an early stage in
order to commence treatment before adverse eects can manifest or
become irreversible.
CMR and MR angiography are excellent tools to evaluate le
ventricular function, associated aortic valve malformation as well as
aortic arch morphology. Residual coarctation or formation of aortic
aneurysms can be detected reliably [58-60] (Figure 2). In addition,
patients with CoA show a high prevalence of associated cardiovascular
abnormalities which can also be detected using CMR imaging
techniques like 3D SSFP sequences [58,61].
CMR imaging in patients with CoA also has high prognostic
value regarding the likelihood of postoperative complications. Late
systemic hypertension or re-coarctation aer surgical repair appear
to be predictable to some extent using aortic arch morphology and
ow measurements [59,62-65]. New imaging techniques such as 4D
ow measurements can be used to evaluate hemodynamic indicators
like vortex formation, vascular strain and collateral-vessel-ow. ese
CMR techniques employed aer surgical repair may help predict
Flow Volume
600
400
200
0
-200
-400
0 10 20 30 40 50 60 70 80 90 100
0 100 200 300 400 500 600 700
[ml/s]
Time [ms]
ROl1: Fo rward flow volume: 98,87 ml 9,46 l/min
Backward flow volume: 62,95 ml 6,02 l/min
Regurgitant fraction: 64%
Regurgitant fraction
C
LPA
RPA
PV+stenosis
D
WL: 309 WW: 338
Figure 1: Tetralogy of Fallot.
A: 4 chamber view cine imaging with normal size of RV and LV. B: Enlarged RV and normal sized LV in a patient with pulmonary regurgitation after repair of TOF with
transanular patch plasty. The apex is formed by the RV (*) while in a normally sized heart, it is formed by the LV. C: Volume change over time from ow measurement
of the pulmonary trunk showing severe pulmonary regurgitation with 64% regurgitant fraction. D: VR 3D Reconstruction of the right ventricle and pulmonary arteries
from MR angiography. Valvular and supravalvular stenosis in the homograft (HO) and stenosis of the RPA (+) with aneurysmatic enlargement of the LPA (*) in a patient
with TOF after corrective surgery with pulmonary valve replacement by homograft.

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 5 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
complications and determine the schedule for follow-up screenings or
interventional therapy [66-68].
In patients with CoA aer interventional therapy dedicated
imaging sequences like gradient echo cine sequences using a high ip
angle can be used to characterize coarctation stents and estimate stent-
associated stenosis using 3D-angiography and phase-contrast ow
mapping [69,70]. Still, depending on the material of the coarctation-
stent used, there are limitations for the assessment of in-stent-stenosis
and morphology using CMR, which may necessitate the alternative use
of cardiovascular CT [71-73].
An interesting future prospect for the use of CMR in patients
with CoA may be personalized 3D hemodanymic simulations based
on CMR data. Possibly, this could help to determine whether a patient
would benet more from either surgery or interventional therapy.
Using computer-generated hemodynamic simulations it would be
possible to compare the changes in simulated hemodynamics aer
either procedure [68,74].
Transposition of the great arteries (TGA)
Transposition of the great arteries TGA is the second most
common cyanotic heart defect aer TOF. It is subdivided into simple
and complex TGA. Depending on the position of the aorta, it is also
subdivided into dextro (D) and levo (L or congenitally corrected (cc))
TGA.
Survival of patients with dTGA is dependent on sucient mixing
of oxygen saturated and depleted blood on the atrial level. is mixing
is augmented by additional shunt connections that increase pulmonary
vascular perfusion and thereby le atrial pressure (i.e. VSD, PDA).
Surgically, patients with dTGA are nowadays corrected by the arterial
switch operation (ASO). e procedure involves reposition of the
great arteries to their corresponding ventricle (LeCompte maneuver),
accompanied by excision and reimplantation of the coronary arteries,
early in life.
e dierent forms of repair/ palliation present a challenge for
the CMR examiner. For post operative follow up CMR can be most
valuable.
TGA aer anatomical correction by arterial switch operation
(Jatene procedure): e arterial switch operation (ASO) can be
associated with stenosis of the pulmonary artery due to the LeCompte
maneuver, supravalvular stenosis of the aorta or pulmonary artery,
aortic root dilatation, aortic valve regurgitation or coronary problems.
Vascular anatomy and valve patency are evaluated using cine
imaging, MR-angiography with 3-D reconstruction and velocity
encoded phase contrast techniques (Figure 3). Coronary morphology
at least in the proximal part can be assessed by MR-angiography, too.
e eect of coronary stenosis on myocardial perfusion can be seen
in sequences involving perfusion stress imaging with adenosine or
dobutamine.
Echocardiography and CMR have been compared in patients
aer arterial switch operation. Echocardiography underestimated RV
function, image quality and visualization of the baes was superior in
CMR [75]. Unbalanced distribution of pulmonary blood ow due to
pulmonary arterial branch stenosis aer ASO appears to be associated
with reduced exercise capacity and increased ventilator drive. CMR can
help in dierentiating the pulmonary artery lesions that are functionally
important [76].
Figure 2: Aortic Coarctation.
A: MR angiography of native coarctation of the aorta in a 9 year old boy with
subtotal stenosis at the level of the aortic isthmus (*) and multiple subclavian
and intercostal collaterals (+) inserting into the descending aorta (DAO) distal
to stenosis (AO= aorta). B: Virtual reality (VR) 3D reconstruction from MR
angiograpy in a 6 year old girl with aortic coarctation (*) and collaterals (+). C:
VR 3D reconstruction from MR angiography after surgical resection of aortic
coarctation and end-to-end anastomosis. Only very slight residual narrowing
at the site of the former stenosis (*). D: VR 3D reconstruction form MR
angiography of re-stenosis (*) after surgical resection of aortic coarction and
end-to-end-anastomosis. AAO = ascending aorta, DAO= descending aorta.
Figure 3: TGA.
TGA after arterial switch operation (ASO) and LeCompte maneuver . The
pulmonary artery (PA) is brought anterior of the ascending aorta (AAO) and the
LPA and RPA “ow” around the AAO. DAO=descending aorta, SVC=superior
caval vein.

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 6 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
TGA aer atrial switch procedure (Mustard or Senning
procedure): Since the 1960’s, patients have been or - if additional
cardiac malformations exsist that make the arterial switch correction
impossible - still are palliated by the atrial switch operation techniques
such as Mustard or Senning procedures. ese involve the creation
of an intraatrial articial tunnel that shunts desaturated blood from
the caval veins to the le sided AV-valve and via the le ventricle to
the pulmonary artery. Saturated blood from the pulmonary veins is
shunted to the right sided AV-valve and via the right ventricle to the
aorta.
e very unusual cardiac anatomy aer atrial switch procedures
can be assessed by cine and 3D-imaging in CMR. e venous bae,
RV- (systemic ventricle) and LV- (sub-pulmonary ventricle) function,
bae leaks or obstructions can be detected by CMR using cine imaging
and inplane ow maps (Figure 4).
For patients late aer Mustard/ Senning procedures, a correlation
between CMR derived RV systolic and diastolic volumes, NT-proBNP
and QRS duration on ECG has been shown [77]. Moreover, compared to
congenitally corrected TGA (ccTGA), dTGA patients aer atrial switch
procedures cannot increase stroke volume during stress [78]. ey also
exhibit impaired ventricular lling as well as decreased contractility in
dobutamine stress CMR [79]. e RV EF, QRS duration and incidence
of arrhythmia have been shown to correlate with LGE detectable scar in
patients aer arterial switch operation and with congenitally corrected
TGA (ccTGA) [14,80]. Function of the pressure unloaded LV (i.e.
sub-pulmonary ventricle) aer atrial switch has also been looked at by
CMR: Decreased ventricular torsion and diastolic abnormalities have
been implied to be measures of subclinical ventricular dysfunction [81].
Aortic function appears to be compromised in patients with TGA and
atrial switch with signicant dilatation of the aortic annulus and the
sinus of Valsalva. e ascending aorta exhibits reduced distensibility in
these patients. No correlation with RV size, function and mass in the
young sample group could be demonstrated [82].
identify stenosis at the anastomosis sites (usually right PA with SVC
and IVC), assess ventricular function and valve patency and evaluate
brosis as a predictive parameter (Figure 5A).
Reduced venous ow in CMR has been linked to suboptimal
Fontan hemodynamics and failing Fontan circulation [83]. Venous
ow and especially its pulsatility has been compared in atrio pulmonary
anastomosis (APA), lateral intracardiac tunnel (LIT) and extracardiac
tunnel (ET) Fontan modications. As could be expected, APA was
associated with more pulsatile ow, but also with increased backow,
atrial enlargement and occurrence of arrhythmia compared to LIT and
ET [84]. Moreover, collateral ow has been identied as a contributor
to enhanced pulmonary ow during stress, while decreased diastolic
compliance is one cause of ventricular dysfunction [85]. Recently,
4D-Flow maps of Fontan circulation have been acquired (see picture
5 B-D) and dierent modications such as the lateral tunnel and the
extracardiac conduit have been compared with this technique. e
dierent modications exhibit dierent ow patterns and formation
of vortices depending on the preceding operative steps (i.e. Glenn or
Hemifontan) [86]. ese studies may help to determine the eect of
ow dynamics - seen also in conventional angiography and evaluated
subjectively – on the long term function of Fontan circulation.
However, further studies are necessary to implement these ndings
into clinical practice. Exercise capacity of Fontan patients is usually
lower than that of healthy children or adults in the corresponding age
group. However, CMR derived myocardial mass and functions do not
correlate very well with BNP levels, patient status, physical exercise
capacity or prognosis [87].
Figure 5: Fontan/ TCPC.
A: Reconstructed MR angiography of a patient with tricuspid atresia after
completion of a total cavo pulmonary connection (TCPC). An extracardiac
conduit (CO) connects the IVC to the right pulmonary artery (RPA), while the
SVC is anastomosed directly to the RPA (Glenn anastomosis). B-D: 4D-Flow
measurement of the Fontan tunnel and Glenn anstomosis (anterior (B), lateral
(C) and posterior (D) views). Blood ow dynamics are visualized by vectors and
blood from the IVC ows preferentially into the RPA (yellow), while the SVC
drains into the LPA (blue). AO= aorta; LPA= left pulmonary artery, SVC/ IVC=
superior/ inferior caval vein.
(B to D: courtesy of Prof. Michael Markl, Director Cardiovascular MR Research,
Northwestern University, Chicago, IL, USA).
Figure 4: TGA.
TGA after atrial switch operation with Mustard procedure. A: The aorta (AO) is
positioned anteriorly and arises from the hypertrophied, morphologically RV.
The pulmonary artery (PA) is positioned posteriorly and arises from the LV,
which is smaller and has less myocardial mass. The septum (*) is pertrudes
into the LV due to increased RV pressure. B: Bafe view in TGA after Mustard
procedure with a bafe from the SVC and IVC to the mitral valve (MV), with
the superior (+) and inferior (*) bafe. C: In plane ow map of the same view.
Fontan circulation/ total cavo pulmonary connection (TCPC)
Aer univentricular palliation following the modied Fontan
principle (TCPC), patients usually have to live with one ventricle
that supplies the systemic circulation. e pulmonary circulation
is maintained passively and aided by a suctioning component of the
ventricle and by the varying intrathoracic pressure during in- and
exspiration. CMR is very helpful in imaging the TCPC anatomy,

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 7 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
Miscellaneous lesions
Anomalous pulmonary venous return: Total and partial
anomalous pulmonary venous return (APVR) can be diagnosed by
CMR. Anatomic details and vessel aberrancy can be shown by MR
angiography and dedicated 3D multiplanar reconstructions (Figure 6).
e magnitude of the shunt volume can be assessed by velocity encoded
ow measurements to quantify pulmonary and systemic blood ow
(Qp and Qs) [88-90].
Recently, attempts have been undertaken to use 4D-velocity
encoded cine MR imaging to improve diagnosis of APVR [91]. As with
other 4D-ow studies, further data is needed to validate the use of this
modality in the clinical setting.
Arrhythmogenic right ventricular cardiomyopathy (ARVC):
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a
rare, but important cause for ventricular tachychardia and sudden
cardiac death in children and young adults [95,96]. ARVC has
been identied as agenetically determined disorder resulting in a
progressing brofatty replacement of right ventricular cardiomyocytes
[97]. e morphological correlate of ARVC is the development of
wall aneurysms and segmental dilatation of the RV. e three typical
sites of dysplasia are the RV apex, the inow portion and the outow
tract (“triangle of dysplasia”) whereas le ventricular involvement is
possible but rare [98]. Due to the progressing nature of the disease,
severity and clinical presentation may vary from unspecic symptoms
like dizziness, palpitation and premature ventricular contractions
(PVCs) to more severe ndings like syncope, right ventricular heart
failure, cardiomegaly and ventricular tachycardia [99]. It is not
uncommon that in a previously healthy young adult the rst and
only documented symptom of ARVC is sudden cardiac death due to
ventricular brillation [100].
CMR is an important diagnostic tool in the diagnosis of ARVC. It
can reliably and noninvasively visualize abnormalities of the ventricular
morphology including increased trabecularization, dilatation or
aneurysm formation [101,102]. Using SSFP cine-sequences with high
spatial and temporal resolution ventricular function and regional
abnormalities in ventricular wall motion, such as akinesia, dyskinesia
or dyssynchronous contraction can be assessed reliably [103,104].
e aforementioned pathological correlates of ARVC in CMR
imaging are essential parts of the Revised Task Force Criteria for
the diagnosis of ARVC that should be analyzed in any patient being
suspected of ARVC [105]. Major CMR imaging criteria are regional
right ventricular akinesia, dyskinesia or aneurysm formation in
combination with either a pathological ratio of RV EDV to BSA (>
110 ml/m2 in males and >100 ml/m2 in females) or a reduced RV EF
<40% [106,107]. Additionally myocardial LGE can be utilized for
tissue characterization. However late enhancement analysis of the
right ventricle can be more dicult than in the le ventricle due to its
thinner walls and possible signal confusion with fat [108].
Hypertrophic cardiomyopathy (HCM)/ Hypertrophyic
obstructive cardiomyopathy (HOCM): For the latest progress in MR
Figure 6: Total anomalous pulmonary venous return.
VR 3D reconstruction from MR angiography. Complex malformation with single
ventricle and total anomalous pulmonary venous return. Drainage of the left
pulmonary veins (PV) into the left SVC (LSVC) and the right PVs into the SVC.
Both right and left SVC are connected by an enlarged anonymous vein (AV). A:
anterior view, B: posterior view.
Figure 7: Ebstein’s Anomaly.
SSFP-Image of an atrialised RV in Ebstein’s anomaly. The very large right
atrium (RA) is connected to the RV by a dysplastic tricuspid valve (TV) that is
displaced towards the apex of the heart (*).
Ebstein’s anomaly: Ebstein’s Anomaly is a combination of an
atrialized portion of the RV, tricuspid valve dysplasia with displacement
of the septal origin of the valve towards the apex of more than 25mm
and RV dysfunction (Figure 7). Few studies have addressed Ebstein’s
Anomaly by CMR and patient numbers are small. CMR is reliably used
for verifying the initial diagnosis as well as follow up of RV function
[92]. In a recent study, CMR derived ventricular and atrial measures
were related to exercise capacity data. Ebstein patients exhibit increased
RV sizes, atrialized portions of 25+/-24 ml/m2 and decreased peak
oxygen uptake (VO2) of 65+/-20% of normal values. e atrialized RV
volume from CMR was related to aerobic capacity and the volume of
the atrialized RV can be used as a measure for the severity of the disease
[93]. Attempts have been undertaken to classify severity of Ebstein’s
Anomaly prenatally by combination of fetal echocardiography and
fetal MRI [94], but further steps are needed to implement this in
clinical routine.
Cardiomyopathy: CMR is increasingly used to identify and
asses risks in patients threatened by sudden cardiac death due to
cardiomyopathies such as hypertrophic obstructive cardiomyopathy
(HOCM) or arrhythmogenic rightventricular cardiomyoapthy
(ARVC).

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 8 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
and HCM/ HOCM, we would like to refer the interested reader to
recent and comprehensive review articles.
Summary
CMR is a very useful tool in the diagnosis, evaluation and
management of patients with congenital heart disease (CHD). For
CHD patients, the whole spectrum of CMR techniques is employed
and delivers valuable information concerning ventricular and valve
function, anatomy of malformed, surgically or interventionally
corrected or palliated hearts and great vessel defects. It helps to correlate
exercise capacity and prognostic factors such as scar formation,
brosis or ventricle size. Depending on the individual defect, CMR
complements other imaging modalities such as echocardiography and
heart catheterization.
New MR techniques are being developed that in future might help
to increase the role of CMR in the clinician’s decision making. Among
these are real time MRI, 3-/4D-Flow measurements, virtual surgery
based on CMR data and the MRI heart catheterization laboratory.
MRI based catheter interventions and measurements are possible in
experimental settings. A future aim is the use of the advantages of CMR
(high resolution, 3 dimensionality, non-x-ray) and combine them with
those of the catheterization laboratory (measurement of pressures,
oxygen saturation, interventions).
Overall, CMR already does play an ever increasing role in the
management of CHD patients.
References
1. Gaca AM, Jaggers JJ, Dudley LT, Bisset GS 3rd (2008) Repair of congenital
heart disease: a primer-part 1. Radiology 247: 617-631.
2. Gaca AM, Jaggers JJ, Dudley LT, Bisset GS 3rd (2008) Repair of congenital
heart disease: a primer--Part 2. Radiology 248: 44-60.
3. Lai WW, Geva T, Shirali GS, Frommelt PC, Humes RA, et al. (2006)
Guidelines and standards for performance of a pediatric echocardiogram: a
report from the Task Force of the Pediatric Council of the American Society of
Echocardiography. J Am Soc Echocardiogr 19: 1413-1430.
4. Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, et al. (2010)
Recommendations for quantication methods during the performance of a
pediatric echocardiogram: a report from the Pediatric Measurements Writing
Group of the American Society of Echocardiography Pediatric and Congenital
Heart Disease Council. J Am Soc Echocardiogr 23: 465-495, quiz 576-577.
5. Ntsinjana HN, Hughes ML, Taylor AM (2011) The Role of Cardiovascular
Magnetic Resonance in Pediatric Congenital Heart Disease. J Cardiovasc
Magn Reson 13: 51.
6. Powell AJ, Geva T (2000) Blood ow measurement by magnetic resonance
imaging in congenital heart disease. Pediatr Cardiol 21: 47-58.
7. van den Bosch AE, Robbers-Visser D, Krenning BJ, Voormolen MM, McGhie
JS, et al. (2006) Real-time transthoracic three-dimensional echocardiographic
assessment of left ventricular volume and ejection fraction in congenital heart
disease. J Am Soc Echocardiogr 19: 1-6.
8. Riesenkampff E, Rietdorf U, Wolf I, Schnackenburg B, Ewert P, et al. (2009)
The practical clinical value of three-dimensional models of complex congenitally
malformed hearts. J Thorac Cardiovasc Surg 138: 571-580.
9. Kilner PJ (2011) The role of cardiovascular magnetic resonance in adults with
congenital heart disease. Prog Cardiovasc Dis 54: 295-304.
10. Debl K, Djavidani B, Buchner S, Heinicke N, Poschenrieder F, et al. (2009)
Quantication of left-to-right shunting in adult congenital heart disease: phase-
contrast cine MRI compared with invasive oximetry. Br J Radiol 82: 386-391.
11. Floemer F, Ulmer HE, Brockmeier K (2000) Images in congenital heart disease.
Use of 3D volume rendered magnetic resonance angiography to demonstrate a
cervical aortic arch. Cardiol Young 10: 423-424.
12. Hunold P, Schlosser T, Vogt FM, Eggebrecht H, Schmermund A, et al. (2005)
Myocardial late enhancement in contrast-enhanced cardiac MRI: distinction
between infarction scar and non-infarction-related disease. AJR Am J
Roentgenol 184: 1420-1426.
13. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, et al. (2006)
Ventricular brosis suggested by cardiovascular magnetic resonance in adults
with repaired tetralogy of fallot and its relationship to adverse markers of clinical
outcome. Circulation 113: 405-413.
14. Babu-Narayan SV, Goktekin O, Moon JC, Broberg CS, Pantely GA, et al.
(2005) Late gadolinium enhancement cardiovascular magnetic resonance of
the systemic right ventricle in adults with previous atrial redirection surgery for
transposition of the great arteries. Circulation 111: 2091-2098.
15. Rathod RH, Prakash A, Powell AJ, Geva T (2010) Myocardial brosis identied
by cardiac magnetic resonance late gadolinium enhancement is associated
with adverse ventricular mechanics and ventricular tachycardia late after
Fontan operation. J Am Coll Cardiol 55: 1721-1728.
16. O’Hanlon R, Grasso A, Roughton M, Moon JC, Clark S, et al. (2010) Prognostic
signicance of myocardial brosis in hypertrophic cardiomyopathy. J Am Coll
Cardiol 56: 867-874.
17. Zemrak F, Petersen SE (2011) Late Gadolinium Enhancement CMR Predicts
Adverse Cardiovascular Outcomes and Mortality in Patients With Coronary
Artery Disease: Systematic Review and Meta-Analysis. Prog Cardiovasc Dis
54: 215-229.
18. Manso B, Castellote A, Dos L, Casaldaliga J (2010) Myocardial perfusion
magnetic resonance imaging for detecting coronary function anomalies in
asymptomatic paediatric patients with a previous arterial switch operation for
the transposition of great arteries. Cardiol Young 20: 410-417.
19. Brix L, Ringgaard S, Rasmusson A, Sorensen TS, Kim WY (2009) Three
dimensional three component whole heart cardiovascular magnetic resonance
velocity mapping: comparison of ow measurements from 3D and 2D
acquisitions. J Cardiovasc Magn Reson 11: 3.
20. Markl M, Geiger J, Stiller B, Arnold R (2011) Impaired continuity of ow in
congenital heart disease with single ventricle physiology. Interact Cardiovasc
Thorac Surg 12: 87-90.
21. Nordmeyer S, Riesenkampff E, Crelier G, Khasheei A, Schnackenburg B, et al.
(2010) Flow-sensitive four-dimensional cine magnetic resonance imaging for
ofine blood ow quantication in multiple vessels: a validation study. J Magn
Reson Imaging 32: 677-683.
22. Pedra CA, Fleishman C, Pedra SF, Cheatham JP (2011) New imaging
modalities in the catheterization laboratory. Curr Opin Cardiol 26: 86-93.
23. Neizel M, Kramer N, Schutte A, Schnackenburg B, Kruger S, et al. (2010)
Magnetic resonance imaging of the cardiac venous system and magnetic
resonance-guided intubation of the coronary sinus in swine: a feasibility study.
Invest Radiol 45: 502-506.
24. Neizel M, Kramer N, Bonner F, Schutte A, Kruger S, et al. (2010) Rapid right
ventricular pacing with MR-compatible pacemaker lead for MR-guided aortic
balloon valvuloplasty in swine. Radiology 255: 799-804.
25. Ratnayaka K, Raman VK, Faranesh AZ, Sonmez M, Kim JH, et al. (2009)
Antegrade percutaneous closure of membranous ventricular septal defect
using X-ray fused with magnetic resonance imaging. JACC Cardiovasc Interv
2: 224-230.
26. Kos S, Huegli R, Hofmann E, Quick HH, Kuehl H, et al. (2009) First magnetic
resonance imaging-guided aortic stenting and cava lter placement using
a polyetheretherketone-based magnetic resonance imaging-compatible
guidewire in swine: proof of concept. Cardiovasc Intervent Radiol 32: 514-521.
27. Kos S, Huegli R, Hofmann E, Quick HH, Kuehl H, et al. (2009) Feasibility of
real-time magnetic resonance-guided angioplasty and stenting of renal arteries
in vitro and in Swine, using a new polyetheretherketone-based magnetic
resonance-compatible guidewire. Invest Radiol 44: 234-241.
28. Kos S, Huegli R, Hofmann E, Quick HH, Kuehl H, et al. (2009) MR-compatible
polyetheretherketone-based guide wire assisting MR-guided stenting of iliac
and supraaortic arteries in swine: feasibility study. Minim Invasive Ther Allied
Technol 18: 181-188.
29. Raval AN, Telep JD, Guttman MA, Ozturk C, Jones M, et al. (2005) Real-
time magnetic resonance imaging-guided stenting of aortic coarctation with
commercially available catheter devices in Swine. Circulation 112: 699-706.
30. Schmitt B, Steendijk P, Lunze K, Ovroutski S, Falkenberg J, et al. (2009)
Integrated assessment of diastolic and systolic ventricular function using

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 9 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
diagnostic cardiac magnetic resonance catheterization: validation in pigs and
application in a clinical pilot study. JACC Cardiovasc Imaging 2: 1271-1281.
31. George AK, Faranesh AZ, Ratnayaka K, Derbyshire JA, Lederman RJ, et al.
(2011) Virtual dye angiography: Flow visualization for MRI-guided interventions.
Magn Reson Med.
32. Saikus CE, Ratnayaka K, Barbash IM, Colyer JH, Kocaturk O, et al. (2011)
MRI-guided vascular access with an active visualization needle. J Magn Reson
Imaging 34: 1159-1166.
33. Wu V, Barbash IM, Ratnayaka K, Saikus CE, Sonmez M, et al. (2011) Adaptive
noise cancellation to suppress electrocardiography artifacts during real-time
interventional MRI. J Magn Reson Imaging 33: 1184-1193.
34. Ratnayaka K, Faranesh AZ, Guttman MA, Kocaturk O, Saikus CE, et al.
(2008) Interventional cardiovascular magnetic resonance: still tantalizing. J
Cardiovasc Magn Reson 10: 62.
35. Moore P (2005) MRI-guided congenital cardiac catheterization and intervention:
the future? Catheter Cardiovasc Interv 66: 1-8.
36. Greil GF, Wolf I, Kuettner A, Fenchel M, Miller S, et al. (2007) Stereolithographic
reproduction of complex cardiac morphology based on high spatial resolution
imaging. Clin Res Cardiol 96: 176-185.
37. Sorensen TS, Beerbaum P, Mosegaard J, Rasmusson A, Schaeffter T, et
al. (2008) Virtual cardiotomy based on 3-D MRI for preoperative planning in
congenital heart disease. Pediatr Radiol 38: 1314-1322.
38. Sorensen TS, Beerbaum P, Mosegaard J, Greil GF (2009) Developing and
evaluating virtual cardiotomy for preoperative planning in congenital heart
disease. Stud Health Technol Inform 142: 340-345.
39. Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, et al.
(2007) Preoperative thresholds for pulmonary valve replacement in patients
with corrected tetralogy of Fallot using cardiovascular magnetic resonance.
Circulation 116: 545-551.
40. Sarikouch S, Peters B, Gutberlet M, Leismann B, Kelter-Kloepping A, et al.
(2010) Sex-specic pediatric percentiles for ventricular size and mass as
reference values for cardiac MRI: assessment by steady-state free-precession
and phase-contrast MRI ow. Circ Cardiovasc Imaging 3: 65-76.
41. Sarikouch S, Koerperich H, Dubowy KO, Boethig D, Boettler P, et al. (2011)
Impact of Gender and Age on Cardiovascular Function Late After Repair of
Tetralogy of Fallot: Percentiles Based on Cardiac Magnetic Resonance. Circ
Cardiovasc Imaging 4: 703-711.
42. Spiewak M, Biernacka EK, Malek LA, Petryka J, Kowalski M, et al. (2011) Right
ventricular outow tract obstruction as a confounding factor in the assessment
of the impact of pulmonary regurgitation on the right ventricular size and
function in patients after repair of tetralogy of Fallot. J Magn Reson Imaging
33: 1040-1046.
43. Park SJ, On YK, Kim JS, Park SW, Yang JH, et al. (2011) Relation of
Fragmented QRS Complex to Right Ventricular Fibrosis Detected by Late
Gadolinium Enhancement Cardiac Magnetic Resonance in Adults With
Repaired Tetralogy of Fallot. Am J Cardiol 109: 110-115.
44. Kempny A, Diller GP, Orwat S, Kaleschke G, Kerckhoff G, et al. (2010) Right
ventricular-left ventricular interaction in adults with Tetralogy of Fallot: A
combined cardiac magnetic resonance and echocardiographic speckle tracking
study. Int J Cardiol 154: 259-264.
45. Riesenkampff E, Mengelkamp L, Mueller M, Kropf S, Abdul-Khaliq H, et al.
(2010) Integrated analysis of atrioventricular interactions in tetralogy of Fallot.
Am J Physiol Heart Circ Physiol 299: H364-371.
46. Morcos P, Vick GW 3rd, Sahn DJ, Jerosch-Herold M, Shurman A (2009)
Correlation of right ventricular ejection fraction and tricuspid annular plane
systolic excursion in tetralogy of Fallot by magnetic resonance imaging. Int J
Cardiovasc Imaging 25: 263-270.
47. Bodhey NK, Beerbaum P, Sarikouch S, Kropf S, Lange P, et al. (2008)
Functional analysis of the components of the right ventricle in the setting of
tetralogy of Fallot. Circ Cardiovasc Imaging 1: 141-147.
48. Iriart X, Montaudon M, Latte S, Chabaneix J, Reant P, et al. (2009) Right
ventricle three-dimensional echography in corrected tetralogy of fallot: accuracy
and variability. Eur J Echocardiogr 10: 784-792.
49. Romeih S, Kroft LJ, Bokenkamp R, Schalij MJ, Grotenhuis H, et al. (2009)
Delayed improvement of right ventricular diastolic function and regression
of right ventricular mass after percutaneous pulmonary valve implantation in
patients with congenital heart disease. Am Heart J 158: 40-46.
50. Jenkins NP, Ward C (1999) Coarctation of the aorta: natural history and
outcome after surgical treatment. Qjm 92: 365-371.
51. Aboulhosn J, Levi DS, Child JS (2011) Common congenital heart disorders in
adults: percutaneous therapeutic procedures. Curr probl Cardiol 36: 263-284.
52. Fruh S, Knirsch W, Dodge-Khatami A, Dave H, Pretre R, et al. (2011)
Comparison of surgical and interventional therapy of native and recurrent aortic
coarctation regarding different age groups during childhood. Eur J Cardiothorac
Surg 39: 898-904.
53. Mohan UR, Danon S, Levi D, Connolly D, Moore JW (2009) Stent implantation
for coarctation of the aorta in children <30 kg. JACC Cardiovascular
interventions 2: 877-883.
54. Tsai SF, Trivedi M, Boettner B, Daniels CJ (2011) Usefulness of screening
cardiovascular magnetic resonance imaging to detect aortic abnormalities after
repair of coarctation of the aorta. Am J Cardiol 107: 297-301.
55. Puranik R, Tsang VT, Puranik S, Jones R, Cullen S, et al. (2009) Late magnetic
resonance surveillance of repaired coarctation of the aorta. Eur J Cardiothorac
Surg 36: 91-95; discussion 95.
56. Mendelsohn AM, Crowley DC, Lindauer A, Beekman RH 3rd (1992) Rapid
progression of aortic aneurysms after patch aortoplasty repair of coarctation of
the aorta. J Am Coll Cardiol 20: 381-385.
57. Piciucchi S, Goodman LR, Earing M, Nicolosi A, Almassi H, et al. (2008) Aortic
aneurysms: delayed complications of coarctation of the aorta repair using
Dacron patch aortoplasty. J Thorac Imaging 23: 278-283.
58. Cantinotti M, Hegde S, Bell A, Razavi R (2008) Diagnostic role of magnetic
resonance imaging in identifying aortic arch anomalies. Congenit Heart Dis 3:
117-123.
59. Hom JJ, Ordovas K, Reddy GP (2008) Velocity-encoded cine MR imaging
in aortic coarctation: functional assessment of hemodynamic events.
Radiographics 28: 407-416.
60. Secchi F, Iozzelli A, Papini GD, Aliprandi A, Di Leo G, et al. (2009) MR imaging
of aortic coarctation. Radiol Med 114: 524-537.
61. Teo LL, Cannell T, Babu-Narayan SV, Hughes M, Mohiaddin RH (2011)
Prevalence of associated cardiovascular abnormalities in 500 patients with
aortic coarctation referred for cardiovascular magnetic resonance imaging to a
tertiary center. Pediatr Cardiol 32: 1120-1127.
62. Ou P, Mousseaux E, Celermajer DS, Pedroni E, Vouhe P, et al. (2006) Aortic
arch shape deformation after coarctation surgery: effect on blood pressure
response. J Thorac Cardiovasc Surg 132: 1105-1111.
63. Ou P, Celermajer DS, Mousseaux E, Giron A, Aggoun Y, et al. (2007) Vascular
remodeling after “successful” repair of coarctation: impact of aortic arch
geometry. J Am Coll Cardiol 49: 883-890.
64. Lashley D, Curtin J, Malcolm P, Clark A, Freeman L (2007) Aortic arch
morphology and late systemic hypertension following correction of coarctation
of aorta. Congenit Heart Dis 2: 410-415.
65. Muzzarelli S, Meadows AK, Ordovas KG, Hope MD, Higgins CB, et al. (2011)
Prediction of Hemodynamic Severity of Coarctation by Magnetic Resonance
Imaging. Am J Cardiol 108: 1335-1340.
66. Hope MD, Meadows AK, Hope TA, Ordovas KG, Saloner D, et al. (2010)
Clinical evaluation of aortic coarctation with 4D ow MR imaging. J Magn
Reson Imaging 31: 711-718.
67. Hope MD, Meadows AK, Hope TA, Ordovas KG, Reddy GP, et al. (2008)
Images in cardiovascular medicine. Evaluation of bicuspid aortic valve and
aortic coarctation with 4D ow magnetic resonance imaging. Circulation 117:
2818-2819.
68. Bock J, Frydrychowicz A, Lorenz R, Hirtler D, Barker AJ, et al. (2011) In vivo
noninvasive 4D pressure difference mapping in the human aorta: phantom
comparison and application in healthy volunteers and patients. Magn Reson
Med 66: 1079-1088.
69. Nordmeyer J, Gaudin R, Tann OR, Lurz PC, Bonhoeffer P, et al. (2010) MRI
may be sufcient for noninvasive assessment of great vessel stents: an in vitro
comparison of MRI, CT, and conventional angiography. AJR Am J Roentgenol
195: 865-871.
70. Rasche V, Oberhuber A, Trumpp S, Bornstedt A, Orend KH, et al. (2011)
MRI assessment of thoracic stent grafts after emergency implantation in multi
trauma patients: a feasibility study. Eur Radiol 21: 1397-1405.

Citation: Steinmetz M, Preus HC, Lotz J (2012) Non-Invasive Imaging for Congenital Heart Disease – Recent Progress in Cardiac MRI. J Clinic
Experiment Cardiol S8:008. doi:10.4172/2155-9880.S8-008
Page 10 of 10
ISSN:2155-9880 JCEC, an open access journalCongenital Heart Disease-Recent Discoveries and InnovationsJ Clinic Experiment Cardiol
71. Sridharan S, Yates R, Taylor AM (2005) Optimizing imaging after coarctation
stenting: the clinical utility of multidetector computer tomography. Catheter
Cardiovasc Interv 66: 420-423.
72. Thanopoulos BV, Douskou M, Giannakoulas G (2010) Multislice computed
tomography after stent implantation for aortic coarctation. Eur Heart J 31: 2270.
73. Iezzi R, Dattesi R, Pirro F, Nestola M, Santoro M, et al. (2011) CT angiography
in stent-graft sizing: impact of using inner vs. outer wall measurements of aortic
neck diameters. J Endovasc Ther 18: 280-288.
74. Coogan JS, Chan FP, Taylor CA, Feinstein JA (2011) Computational uid
dynamic simulations of aortic coarctation comparing the effects of surgical-
and stent-based treatments on aortic compliance and ventricular workload.
Catheter Cardiovasc Interv 77: 680-691.
75. Ho JG, Cohen MD, Ebenroth ES, Schamberger MS, Cordes TM, et al. (2011)
Comparison between Transthoracic Echocardiography and Cardiac Magnetic
Resonance Imaging in Patients Status Post Atrial Switch Procedure. Congenit
Heart Dis.
76. Giardini A, Khambadkone S, Taylor A, Derrick G (2010) Effect of abnormal
pulmonary ow distribution on ventilatory efciency and exercise capacity after
arterial switch operation for transposition of great arteries. Am J Cardiol 106:
1023-1028.
77. Plymen CM, Hughes ML, Picaut N, Panoulas VF, Macdonald ST, et al. (2010)
The relationship of systemic right ventricular function to ECG parameters and
NT-proBNP levels in adults with transposition of the great arteries late after
Senning or Mustard surgery. Heart 96: 1569-1573.
78. Fratz S, Hager A, Busch R, Kaemmerer H, Schwaiger M, et al. (2008) Patients
after atrial switch operation for transposition of the great arteries can not
increase stroke volume under dobutamine stress as opposed to patients with
congenitally corrected transposition. Circ J 72: 1130-1135.
79. Tulevski, II, van der Wall EE, Groenink M, Dodge-Khatami A, Hirsch A, et
al. (2002) Usefulness of magnetic resonance imaging dobutamine stress in
asymptomatic and minimally symptomatic patients with decreased cardiac
reserve from congenital heart disease (complete and corrected transposition of
the great arteries and subpulmonic obstruction). Am J Cardiol 89: 1077-1081.
80. Giardini A, Lovato L, Donti A, Formigari R, Oppido G, et al. (2006) Relation
between right ventricular structural alterations and markers of adverse clinical
outcome in adults with systemic right ventricle and either congenital complete
(after Senning operation) or congenitally corrected transposition of the great
arteries. Am J Cardiol 98: 1277-1282.
81. Pettersen E, Lindberg H, Smith HJ, Smevik B, Edvardsen T, et al. (2008) Left
ventricular function in patients with transposition of the great arteries operated
with atrial switch. Pediatr Cardiol 29: 597-603.
82. Ladouceur M, Kachenoura N, Lefort M, Redheuil A, Bonnet D, et al. (2011)
Structure and function of the ascending aorta in palliated transposition of the
great arteries. Int J Cardiol.
83. Ovroutski S, Nordmeyer S, Miera O, Ewert P, Klimes K, et al. (2011) Caval ow
reects Fontan hemodynamics: quantication by magnetic resonance imaging.
Clin Res Cardiol.
84. Klimes K, Abdul-Khaliq H, Ovroutski S, Hui W, Alexi-Meskishvili V, et al. (2007)
Pulmonary and caval blood ow patterns in patients with intracardiac and
extracardiac Fontan: a magnetic resonance study. Clin Res Cardiol 96: 160-
167.
85. Schmitt B, Steendijk P, Ovroutski S, Lunze K, Rahmanzadeh P, et al. (2010)
Pulmonary vascular resistance, collateral ow, and ventricular function in
patients with a Fontan circulation at rest and during dobutamine stress. Circ
Cardiovasc Imaging 3: 623-631.
86. Sundareswaran KS, Haggerty CM, de Zelicourt D, Dasi LP, Pekkan K, et al.
(2011) Visualization of ow structures in Fontan patients using 3-dimensional
phase contrast magnetic resonance imaging. J Thorac Cardiovasc Surg.
87. Atz AM, Zak V, Breitbart RE, Colan SD, Pasquali SK, et al. (2011) Factors
associated with serum brain natriuretic peptide levels after the Fontan
procedure. Congenit Heart Dis 6: 313-321.
88. Riesenkampff EM, Schmitt B, Schnackenburg B, Huebler M, Alexi-Meskishvili
V, et al. (2009) Partial anomalous pulmonary venous drainage in young
pediatric patients: the role of magnetic resonance imaging. Pediatr Cardiol 30:
458-464.
89. Dellegrottaglie S, Pedrotti P, Pedretti S, Mauri F, Roghi A (2008) Atrial septal
defect combined with partial anomalous pulmonary venous return: complete
anatomic and functional characterization by cardiac magnetic resonance. J
Cardiovasc Med (Hagerstown) 9: 1184-1186.
90. Beerbaum P, Parish V, Bell A, Gieseke J, Korperich H, et al. (2008) Atypical
atrial septal defects in children: noninvasive evaluation by cardiac MRI. Pediatr
Radiol 38: 1188-1194.
91. Nordmeyer S, Berger F, Kuehne T, Riesenkampff E (2011) Flow-sensitive four-
dimensional magnetic resonance imaging facilitates and improves the accurate
diagnosis of partial anomalous pulmonary venous drainage. Cardiol Young 21:
528-535.
92. Yalonetsky S, Tobler D, Greutmann M, Crean AM, Wintersperger BJ, et al.
(2011) Cardiac magnetic resonance imaging and the assessment of ebstein
anomaly in adults. Am J Cardiol 107: 767-773.
93. Tobler D, Yalonetsky S, Crean AM, Granton JT, Burchill L, et al. (2011) Right
heart characteristics and exercise parameters in adults with Ebstein anomaly:
New perspectives from cardiac magnetic resonance imaging studies. Int J
Cardiol.
94. Nathan AT, Marino BS, Dominguez T, Tabbutt S, Nicolson S, et al. (2010)
Tricuspid valve dysplasia with severe tricuspid regurgitation: fetal pulmonary
artery size predicts lung viability in the presence of small lung volumes. Fetal
Diagn Ther 27: 101-105.
95. Hamilton RM (2009) Arrhythmogenic right ventricular cardiomyopathy. Pacing
Clin Electrophysiol 32 Suppl 2: S44-51.
96. Sen-Chowdhry S, Morgan RD, Chambers JC, McKenna WJ (2010)
Arrhythmogenic cardiomyopathy: etiology, diagnosis, and treatment. Annu Rev
Med 61: 233-253.
97. Azaouagh A, Churzidse S, Konorza T, Erbel R (2011) Arrhythmogenic right
ventricular cardiomyopathy/dysplasia: a review and update. Clin Res Cardiol
100: 383-394.
98. Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, et al. (1982)
Right ventricular dysplasia: a report of 24 adult cases. Circulation 65: 384-398.
99. McRae AT 3rd, Chung MK, Asher CR (2001) Arrhythmogenic right ventricular
cardiomyopathy: a cause of sudden death in young people. Cleveland Clinic
journal of medicine 68: 459-467.
100. Corrado D, Basso C, Thiene G, McKenna WJ, Davies MJ, et al. (1997)
Spectrum of clinicopathologic manifestations of arrhythmogenic right
ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol
30: 1512-1520.
101. Bluemke DA, Krupinski EA, Ovitt T, Gear K, Unger E, et al. (2003) MR Imaging
of arrhythmogenic right ventricular cardiomyopathy: morphologic ndings and
interobserver reliability. Cardiology 99: 153-162.
102. Tandri H, Daya SK, Nasir K, Bomma C, Lima JA, et al. (2006) Normal
reference values for the adult right ventricle by magnetic resonance imaging.
Am J Cardiol 98: 1660-1664.
103. Maksimovic R, Ekinci O, Reiner C, Bachmann GF, Seferovic PM, et al. (2006)
The value of magnetic resonance imaging for the diagnosis of arrhythmogenic
right ventricular cardiomyopathy. European radiology 16: 560-568.
104. Tandri H, Macedo R, Calkins H, Marcus F, Cannom D, et al. (2008) Role
of magnetic resonance imaging in arrhythmogenic right ventricular dysplasia:
insights from the North American arrhythmogenic right ventricular dysplasia
(ARVD/C) study. Am Heart J 155: 147-153.
105. Sen-Chowdhry S, Prasad SK, Syrris P, Wage R, Ward D, et al. (2006)
Cardiovascular magnetic resonance in arrhythmogenic right ventricular
cardiomyopathy revisited: comparison with task force criteria and genotype. J
Am Coll Cardiol 48: 2132-2140.
106. Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, et al. (2010) Diagnosis
of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed
modication of the task force criteria. Circulation 121: 1533-1541.
107. McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist
C, et al. (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/
cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial
Disease of the European Society of Cardiology and of the Scientic Council on
Cardiomyopathies of the International Society and Federation of Cardiology.
Br Heart J 71: 215-218.
108. Tandri H, Saranathan M, Rodriguez ER, Martinez C, Bomma C, et al. (2005)
Noninvasive detection of myocardial brosis in arrhythmogenic right ventricular
cardiomyopathy using delayed-enhancement magnetic resonance imaging. J
Am Coll Cardiol 45: 98-103.