European Heart Journal Cardiovascular Imaging (2014) 15, 617

doi:10.1093/ehjci/jet124

REVIEW

Imaging of congenital heart disease in adults:

choice of modalities
Stefan Orwat, Gerhard-Paul Diller, and Helmut Baumgartner*
Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Albert-Schweitzer-Str. 33, Muenster 48149, Germany
Received 4 April 2013; accepted after revision 11 June 2013; online publish-ahead-of-print 2 August 2013

----------------------------------------------------------------------------------------------------------------------------------------------------------Keywords

multimodality imaging adult congenital heart disease cardiovascular magnetic resonance echocardiography
computed tomography

Introduction
As the majority of infants born with congenital heart disease (CHD)
now survive into adulthood, lifelong follow-up at specialized centres
is an integral part of their ongoing care. Adequate management of this
group of adult congenital heart disease (ACHD) patients requires
advanced imaging to assess the morphology and function of heart
and vessels. In recent years, strategies for assessing the anatomy
and physiology of CHD have evolved rapidly, with a shift from
cardiac catheterization to non-invasive modalities such as echocardiography and more recently, cardiovascular magnetic resonance
imaging (CMR) and cardiac computed tomography (CT).1 It is particularly important to determine the most appropriate and costeffective diagnostic pathways for ACHD patients and the use of any
imaging method has to be carefully considered.
Since the majority of cardiac defects are nowadays diagnosed in
infancy and childhood, the adult cardiologist is mostly faced with
patients known to have CHD. Nevertheless, the diagnosis may be incomplete or sometimes incorrect and has to be re-established in
adulthood. Therefore, a comprehensive understanding of cardiac
anatomy is required and particularly for describing complex malformations a structured segmental approach is recommended.2

Because of altered anatomy and physiology essential differences

exist between paediatric patients with CHD and most adults with
previously diagnosed or repaired congenital heart defects. In addition, different imaging modalities are available and measurements
cannot be used interchangeable. For example, CT tends to overestimate and echocardiography to underestimate right ventricular (RV)
volumes in comparison with the gold standard CMR.3
The aim of this paper was to describe the current armamentarium
of imaging tools available to the ACHD cardiologist and to develop a
structured approach to imaging in CHD. Specific emphasis is placed
on the current role and future potential of advanced imaging modalities. Selected cardiac lesions will be addressed in more detail.

Strengths and limitations

of currently available imaging
modalities
An overview of strengths and limitations of available imaging modalities is presented in Table 1.

* Corresponding author. Tel: +49 251 83 46110; Fax: +49 251 83 46109, Email: helmut.baumgartner@ukmuenster.de
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2013. For permissions please email: journals.permissions@oup.com

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Major advances in noninvasive imaging of adult congenital heart disease have been accomplished. These tools play now a key role in comprehensive diagnostic work-up, decision for intervention, evaluation for the suitability of specific therapeutic options, monitoring of interventions and
regular follow-up. Besides echocardiography, magnetic resonance (CMR) and computed tomography (CT) have gained particular importance.
The choice of imaging modality has thus become a critical issue. This review summarizes strengths and limitations of the different imaging
modalities and how they may be used in a complementary fashion. Echocardiography obviously remains the workhorse of imaging routinely
used in all patients. However, in complex disease and after surgery echocardiography alone frequently remains insufficient. CMR is particularly
useful in this setting and allows reproducible and accurate quantification of ventricular function and comprehensive assessment of cardiac
anatomy, aorta, pulmonary arteries and venous return including complex flow measurements. CT is preferred when CMR is contraindicated,
when superior spatial resolution is required or when metallic artefacts limit CMR imaging.
In conclusion, the use of currently available imaging modalities in adult congenital heart disease needs to be complementary. Echocardiography remains
the basis tool, CMR and CT should be added considering specific open questions and the ability to answer them, availability and economic issues.

Imaging of congenital heart disease in adults

Table 1

Overview of strengths and limitations of available imaging modalities in ACHD

Modality

Strengths

Limitations

First-line non-invasive imaging technique; high temporal

resolution; real-time imaging capability; superior for
intra-cardiac structures, hemodynamic assessment, and
velocity measurement.
Superior in assessing valve disease and septal defects,
estimating gradients, PA pressure, and detecting highly
mobile structures such as vegetation.

Highly user dependent; access to the heart limited by acoustic

windows; extracardiac anatomy difficult to visualize.

Superior for imaging posterior cardiac structures, monitoring

during interventional and surgical procedures; high
sensitivity for the detection of thrombi and vegetations.
Feasible and safe in CHD, enables assessment of perfusion
reserve.
Two methods: Doppler tissue imaging (DTI) and
speckle-tracking echocardiography (STE), evolving
technology to assess ventricular function in ACHD patients.

Usually requires sedation orfor monitoring of

interventionsgeneral anaesthesia; limited access to
extracardiac structures.
Clinical significance in ACHD still unknown.

New way to assess valve morphology and motion and to

measure ventricular volumes.

Underestimates ventricular volume and function in

comparison with the gold standard.

...............................................................................................................................................................................
Echocardiography
General

2-D echo and

Doppler
TEE

Stress-echo
Deformation imaging

3D-TTE/TEE
Intracardiac echo

May be helpful in guiding percutaneous interventional

procedures, no need for sedation or oesophageal
intubation.
Cardiac magnetic resonance imaging

Limited acoustic window to assess the hole RV and pulmonary

arteries/veins, the aorta beyond its proximal ascending part
and the arch.

Vendor-specific solutions and therefore lack of industry

standardization, DTI is angle dependent.

Large shaft size, only monoplane image sections, no defined

standard views, possible right heart catheterization
complications, high costs.

Unrestricted access/views; coverage of the hole thorax; no

ionizing radiation; superior for extracardiac structures,
good temporal resolution, good assessment of flow; can be
used in pregnancy when clinically indicated (some centres
avoid imaging during the first trimester).

SSFP acquisitions

Gold standard for the assessment of biventricular function and

quantification of volumes (especially RV ejection fraction in
ToF and systemic RV; evaluation of the RVOTO and RV-PA
conduits, PAs, aorta, systemic and pulmonary veins;
quantification of myocardial mass (LV and RV), highly
reproducible measurements.

Deformation imaging

Different techniques: e.g. Tagging-techniques and feature

tracking.

Only a few studies in ACHD patients.

Angiography

Evaluation of pulmonary arteries and the aorta; evaluation of

systemic and pulmonary veins (anomalous connection,
obstruction, etc.).
Measurements of flow volumes, quantification of pulmonary
and aortic regurgitation, quantification of shunts.

Gadolinium-based contrast media rarely cause allergic

reaction or nephrogenic systemic fibrosis in patients with
severe renal dysfunction.
Lower temporal resolution than for continuous-wave
Doppler velocity measurement, dependent on a
homogenous magnetic field; background offset error may
occur.
See angiography; no detection of diffuse myocardial fibrosis.

Flow (phase-contrast
velocity mapping)

LGE
4-D velocity imaging

T1 mapping

Detection and quantification of focal myocardial fibrosis/scar;

tissue characterization (fibrosis, fat, iron, etc.).
Visualization of multidirectional blood flow in a 3-D volume,
pathline visualization may be helpful in selected ACHD
patients.

Long-acquisition time; large resulting datasets need time

consuming post-processing.

Quantitative imaging of extracellular volume fraction,

detection of diffuse fibrosis.

Still no standardized technique and protocol, prognostic

relevance currently unknown.

Excellent spatial resolution; fast acquisition time; alternative to

CMR in patients with pacemaker or implantable
cardioverter defibrillator.

Ionizing radiation; inferior temporal resolution compared with

CMR; fair tissue characterization and hemodynamic
assessment, costly.

Computed tomography
General

Continued

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Sometimes limited tolerability due to claustrophobia, patient

cooperation is required (breath-hold instructions); most
implanted cardiac devices are not tested to be MR
conditional; susceptibility to artefacts caused by metallic
surgical material, loop recorder, coils, or stents can make
measurement impossible; time consuming, expertise
during scanning needed; expensive; availability may be
limited.
ECG-gated cine images and flow maps are typically acquired
over a breath-hold and not in real time. Thin mobile
structures may not be well seen (endocarditis).

General

S. Orwat et al.

Table I Continued
Modality

Strengths

Limitations

Visualization of stented structures, coronaries, collateral

arteries, surgical shunts, arteriovenous malformations, and
coronary artery disease.
Ventricular size and function.

Potential complication: contrast allergy and renal failure.

...............................................................................................................................................................................
Angio

ECG-gated cine CT

Lower temporal resolution than CMR.

Emerging techniques are in italic font.

ECG, electrocardiogram; TTE, transthoracic echocardiography; LV, left ventricle; RV, right ventricle; LGE, late gadolinium enhancement; ToF, tetralogy of Fallot; RVOTO, right
ventricular outflow obstruction; SSFP, steady-state free precession; PA, pulmonary artery.

Echocardiography

Cardiac magnetic resonance imaging

In the last decade, CMR has gained particular importance in the morphological and functional assessment of ACHD, particularly in
patients with complex malformations. The unrestricted views, free
choice of imaging planes, wide range of imaging sequences, and the
lack of ionizing radiation make this technique ideal for the serial assessment of ACHD patients.
To extract optimal clinical information from the scan, however, a
thorough understanding of the underlying anatomy and pathophysiology as well as associated or residual lesions prior to a CMR scan is
required. In most of the cases, CMR imaging is tailored to address
diagnostic questions that have been incompletely addressed by echocardiography.
For data acquisition and analysis, a standard approach should be
applied to ensure that measurements are reproducible and accurate
between patients.18 The European Society of Cardiology (ESC)
recommends standardized CMR protocols for the use in ACHD
patients as published recently.19
The standard technique used for cine images is steady-state free
precession (SSFP) imaging. It is well suited for imaging ventricular
function and is the gold standard for the serial assessment of both
right and left ventricular volumes and function.3,20 23
Beside CMR taggingas an established technique for measuring
regional myocardial function24CMR myocardial feature tracking
is a novel but promising method that detects myocardial borders
and tracks myocardial motion at the voxel level from standard
SSFP cine acquisitions.25 27 Recent studies, even in ACHD patients,
showed good inter-observer reproducibility for measures of global
left and RV strains, suggesting that the approach could have clinical
relevance and deserves further study.28,29 (Figure 1)
CMR imaging has evolved to provide increasingly precise functional analysis of flow dynamics mainly by the use of phase-contrast
sequences.30 In the assessment of an incompetent valve by calculating
regurgitant volume and fraction, velocity-encoded CMR is the
gold standard.31 34 Shunt quantification based on pulmonary and
systemic flow estimates (Qp:Qs) can be performed by plane
velocity-encoded imaging through the main PA and aorta.35,36
Studies in different ACHD pathologies have shown the potential of
four-dimensional (4D) phase-contrast velocity mapping of distorted

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Due to its relatively low cost, good safety profile, and wide availability,
transthoracic echocardiography (TTE) remains the first-line tool for
the regular assessment of ACHD. Two-dimensional TTE together
with colour and spectral Doppler indeed allows the accurate and
comprehensive assessment of cardiac morphology as well as function
in most ACHD patients. This includes basic cardiac anatomy with
orientation and position of the heart, venous return, connection of
the atria and ventricles and origin of the great arteries, the morphology of the heart chambers, ventricular function and shunt connections, as well as morphology and function of heart valves. Doppler
echocardiography provides information on the severity of obstructive lesions, RV/pulmonary artery (PA) pressures from tricuspid
regurgitant velocity and allows to semiquantitatively assess valvular
regurgitation and shunt lesions.
For certain indications such as evaluation of the aorta, pulmonary
venous return, the interatrial septum, or left atrial appendage, transoesophageal echocardiography (TOE) is of particular advantage.
Besides the problems with image quality in patients after surgery or
with abnormal position of the heart in the chest, echocardiography
has some additional limitations: assessment of ventricular volumes
and function remains unsatisfying in patients with systemic and nonsystemic right ventricles and univentricular hearts particularly
because of the unique geometry.4 6 Furthermore, Doppler gradients
may sometimes be misleading particularly in RV outflow tract obstruction (RVOTO), coarctation, and stenosis occurring in series.
Finally, the assessment of venous return and the great arteries
often requires additional imaging modalities.
Real-time three-dimensional (3-D) echocardiography in patients
with CHD is increasingly used7,8 and represents a sensitive tool to
identify left ventricular (LV) and RV dysfunction in this patient
cohort.9
Evolving echocardiographic technologies such as angle-independent
speckle-tracking echocardiography (STE) have been introduced lately
in the evaluation of cardiac function in ACHD. Longitudinal ventricular
dysfunction may precede systolic dysfunction as assessed by conventional parameters and thus, may be more sensitive in detecting myocardial impairment before it becomes evident with conventional imaging
modalities. In fact, STE is increasingly used for the quantification of
cardiac function in ACHD and has the potential to predict adverse clinical outcomes.10 12 However, the lack of standardization and differences between measurements obtained with different machines

limits comparability between different studies and has so far hampered

clinical uptake of the technique.13 17

Imaging of congenital heart disease in adults

Figure 1: CMR feature tracking based assessment of the RV and LV in a patient with tetralogy of Fallot in a four-chamber view (A) and in the midventricular short axis (B).

Computed tomography
Multidetector CT provides excellent spatial resolution and rapid acquisition time and therefore, plays an increasing role in the assessment of cardiac and pulmonary structures in ACHD patients.42 44
Besides its role in the assessment of the coronary artery anatomy,
CT allows for the assessment of biomedical devices such as stents,
valves, and sternal wires. CT scanning of the aorta is a quick and
widely available imaging modality. Retrospectively gated cardiac CT
angiography studies create the images needed for the assessment
of ventricular function and volume. This provides a 4-D volumetric
dataset for the analysis, which showed a good correlation but slight
overestimation to CMR results.3 Tracheobronchial pathology is
often associated in CHD, and can also be nicely evaluated by CT.
Recent developments aim to establish a role for CT in the functional imaging of the heart beyond the mere visualization of anatomy.45
Newer techniques to reduce the radiation dose, such as electrocardiogram (ECG)-controlled dose modulation, ECG-triggered
sequential CT, low kV scanning, and interactive reconstruction,
allow cardiac imaging at a much lower radiation dose.46,47 Despite

these recent developments, adults with CHD often need repetitive

imaging, exposing them to repeated ionizing radiation.

Chest X-ray
Chest X-ray is helpful for serial comparison of heart size, exclusion of
cardiac malposition, and evaluation of pulmonary vascularity, thoracic skeleton, as well as peripheral lung fields.1 It has also been
shown to provide prognostic information in ACHD patients.48
In some centres, it is still routinely performed; however, other
imaging modalities avoiding radiation exposure are increasingly
used and have largely replaced chest radiograph at our and many
other centres.

Cardiac catheterization
Invasive diagnostic studies should only be undertaken once other
non-invasive imaging modalities have been used exhausted. It is
then primarily required for hemodynamic assessment rather than
imaging. Specific indications for invasive assessment remain the measurement of pulmonary vascular resistance, the evaluation of coronary arteries, and collateral vessels. Invasive assessment of systolic
and end-diastolic ventricular function, pressure gradients, as well as
shunts remains reserved for patients in whom non-invasive evaluation leaves uncertainty and the information is critical for clinical
decision-making.
All the above-mentioned imaging techniques require special expertise in complex CHD. Imaging by CT and CMR scanning should
be physician-supervised.

General recommendations for the

choice between echocardiography,
CMR, and CT when imaging ACHD
in clinical practice
(Adapted from the ESC guidelines on the management of grown-up
CHD1). These recommendations are by and large-based expert
opinion due to a lack of randomized and prospective data.

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blood flow patterns in the heart and adjacent large vessels, suggesting
possible roles of fluid dynamic factors in the initiation or progression
of pathology.37 39 The ability to measure multidirectional flows
throughout a study volume has provided novel insights into cardiovascular blood flow patterns.
Contrast-enhanced MR angiography is particularly useful in the
setting of ACHD. Visualization of anomalous pulmonary or systemic
venous return or assessment and illustration of aortic pathology, such
as coarctation and associated collaterals, can be performed.
Myocardial late gadolinium enhancement (LGE) detects myocardial scar and has been used to demonstrate fibrosis in different
groups of ACHD. The finding of LGE is generally associated with
worse functional (New York Heart Association) class, adverse ventricular mechanics, and history of arrhythmias. However, LGE provides only an incomplete estimate of the degree of fibrosis as it
relies on a difference in signal intensity that may not exist in the
case of interstitial myocardial fibrosis.40 CMR T1 mapping is a promising modality for the evaluation of diffuse myocardial fibrosis, and
pilot studies in ACHD patients have already been conducted.41

10

S. Orwat et al.

(1) Echocardiography is always the first modality to be used with

the attempt to answer all questions required for patient
management.
(2) CMR (CT) is indicated as an alternative to echocardiography, if
both techniques can provide similar information but echocardiography cannot be obtained with sufficient quality to provide all
information essential for patient management.
(3) CMR is indicated as a second method if echocardiography measurements of parameters critical for the clinical decisions (i.e.
intervention) are borderline or ambiguous. These indications
include
(i) Ventricular volumes and ejection fraction (particularly in
regurgitant and shunt lesions).
(ii) Severity of regurgitant lesions.
(iii) Severity of shunt lesions.
(4) CMR or CT is indicated in settings where they are considered
superior to echocardiography and should then be regularly
used when the information is essential for patient management.
For the choice between CMR and CT, several aspects must be
considered including radiation exposure, resolution required,
sum of information required, presence of pacemaker/ICD, or
other implants. These indications include:

Specific considerations in selected

lesions generally encountered after
repair during childhood
Tetralogy of Fallot
Despite excellent long-term survival and quality of life after
repair in the current era, residua and sequela can cause long-term
complications in patients with repaired ToF.49,50 Thus, lifelong
regular follow-up after surgery is required. Chronic PR is a very
common problem and may lead to progressive RV dilatation,
increased RV wall stress, and RV dysfunction resulting in a substrate

Table 2 Pre-PVR threshold values of RV end-diastolic

volume index (EDVi) and RV end-systolic volume index
(ESVi) measured by CMR predicting volume
normalization after PVR
N

EDVi (mL/m2)

ESVi (mL/m2)

Buechel et al.57
Therrien et al.56

20
17

,150
,170

na
,85

Dave et al.98

39

,150

na

Oosterhof et al.99
Frigiola et al.100

71
71

,160
,150

,82
na

Geva et al.101

64

na

,90

Lee et al.102

67

,163

,80

................................................................................

na, not available.

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(i) Quantification of RV volumes and ejection fraction (tetralogy of Fallot [ToF], systemic RV, and tricuspid regurgitation) [CMR].
(ii) Evaluation of the RV outflow tract and RV-PA conduits
[CMR and CT].
(iii) Quantification of pulmonary regurgitation (PR) [CMR].
(iv) Evaluation of pulmonary arteries (stenosis and aneurysms)
and the aorta (aneurysm, dissection, and coarctation)
[CMR and CT].
(v) Evaluation of systemic and pulmonary veins (anomalous
connection, obstruction, etc.) [CMR and CT].
(vi) Collaterals and arteriovenous malformations (CT is superior to CMR).
(vii) Coronary anomalies and coronary artery disease (CT is
superior to CMR).
(viii) Evaluation of intra- and extracardiac masses [CMR and
CT].
(ix) Quantification of myocardial mass [CMR and CT].
(x) Detection and quantification of myocardial fibrosis/scar
(gadolinium late enhancement) [CMR].
(xi) Tissue characterization (fibrosis, fat, iron etc.) [CMR].

for potentially malignant arrhythmias, and sudden death. As a consequence of RV dysfunction and inter-ventricular interaction, LV dysfunction may also occur and represents an adverse prognostic
feature in itself.11,51 53
TTE as the first-line diagnostic technique for follow-up provides
comprehensive information on residual RVOTO, the presence and
degree of PR, residual ventricular septal defect, RV and LV size, as
well as systolic and diastolic functions. Two recent studies found a
high degree of correlation between indexed apical RV diastolic
area and indexed CMR end-diastolic volume.54,55 In addition, the
degree of tricuspid regurgitation, RV pressure (RVP), aortic root
size, and aortic regurgitation can be assessed by echocardiography.
Despite its prognostic importance, assessment of PR remains difficult. The treatment for PR is pulmonary valve replacement (PVR),
but its optimal timing in asymptomatic patients, which is based
on clinical, electrocardiographic, and volumetric measurements
remain insufficiently defined.56,57 Although pre-PVR threshold
values have been identified for RV dilation beyond that normalization
of RV size is unlikely after PVR (Table 2), they do not provide ideal criteria for the timing of intervention. Normalization of RV size does not
necessarily translate into normalization of RV function andmore
importantlyimprovement of exercise capacity indicating that intervention for PR may be required even earlier. In addition, dynamic
changes of RV size and function may be more important than single
measurements. Thus, regular follow-up is essential.
Some patients present with restrictive RV physiology resulting in
less RV dilation despite severe pulmonary valve incompetence and
better clinical tolerance of PR. Restrictive RV filling can be identified
by the presence of end-diastolic forward flow in the PA during atrial
contraction indicating diastolic RV stiffness.58 60 (Figure 2)
Myocardial deformation parameters were found to be useful to
detect early myocardial dysfunction in TOF patients.61 63 Nevertheless, echocardiographic assessment of RV volumes and function as
well as PR remain limited by the complex RV geometry, and CMR
is superior in quantifying both.64 Thus, CMR plays a key role in the
diagnostic work-up after repair of ToF and is recommended during
the routine follow-up of these patients.21,65 68 It allows for highly reproducible quantification of RV volumes, especially if performed

Imaging of congenital heart disease in adults

11

Figure 2: Continuous-wave Doppler (A) and CMR flow (B) profiles in the main PA in a patient with repair of tetralogy of Fallot. Note late diastolic
antegrade flow (arrows) as a possible marker for restrictive RV diastolic function.

Transposition of the great arteries

Transposition of the great arteries (TGA) is characterized by the
origin of the great arteries from the morphologically inappropriate
ventricles, while there is a normal connection between the atria
and the ventricles. Many current ACHD patients will have had surgical correction with an atrial switch procedure (Senning or Mustard
operation) to redirect the blood and provide physiological repair.
In addition to arrhythmias, systolic dysfunction of the systemic
(subaortic) RV is the most frequent and relevant clinical problem.73
Quantifying RV function (particularly subclinical RV dysfunction) is
challenging, however. Different echocardiography parameters have
been proposed to assess RV function. These include visual qualitative
assessment (eyeballing, preferred in many centres), measuring RV

fractional area change, tricuspid annular plain systolic excursion,

myocardial performance index, rate of systolic RVP increase (dp/
dt) measured across the tricuspid regurgitant jet, as well as more
recently strain and strain rate-based measures.6,74 76 In this patient
cohort, reduced biventricular longitudinal strain is related to
subpulmonary ventricular function, and relates to adverse clinical
outcome.12
However, despite these advances in echocardiographic technology, CMR assessment of the systemic RV function and volumes
with multislice axial b-SSFP imaging remains the gold standard.19
Tricuspid regurgitation is primarily related to the deteriorating
systemic RV function. Its quantification is frequently difficult by echocardiography and CMR using volumetric measurements may add in
this context.
The intra-atrial tunnels (baffles) can leak with either L R or RL
shunt, or can develop obstruction to systemic venous and/or pulmonary venous drainage. Echocardiography (TTE and TOE) with
the use of Doppler techniques and contrast media may provide this
information, but the evaluation remains frequently incomplete. In
particular, obstruction of the superior vena cava (a frequent site of
baffle stenosis) is difficult to image in adults. CMR spin-echo sequences
in combination with magnetic resonance angiography provide excellent visualization of systemic and pulmonary venous atrial pathways,77
although CT and invasive angiography deliver the highest resolution
(Figure 3).
In the past three decades, repair by the arterial switch operation
has become the method of choice providing not only physiological
but also anatomical repair with the morphologic LV serving as systemic ventricle and avoidance of the surgical trauma at the atrial
level with all its consequences. The most common long-term problems are PA and PA branch stenosis (in part related to PA-stretching
after LeCompte manoeuvre), dilatation of the proximal neo-aorta
accompanied by aortic regurgitation, and less commonly coronary
artery pathology causing myocardial ischaemia.78,79 CMR is ideal
for imaging the aorta, branch pulmonary arteries, and RVOT, the
main sites of concern. Flow measurement can reveal a distributional
flow imbalance between the left and right lungs. Echocardiographic

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from datasets in axial orientation using manual blood-myocardial delineation.69 A recent study by Wald et al.70 concluded that indexed PR
volume rather than PR fraction may be a more sensitive and accurate
measure of RV volume load and thus, may better describe the physiologic significance of PR. Using phase-contrast analysis of the main PA
flow or analysis of differential right and left ventricular stroke
volumes, PR can be quantified by providing regurgitant volume and
regurgitant fraction.
CMR should also routinely be used for the evaluation of RVOTO,
the pulmonary arteries to assess their size and shape and exclude
stenosis, the ascending aorta, and the position of great vessels or conduits in relation to the sternum (essential when planning reoperation
with sternotomy). Although the rate of aortic growth seems to be
slow, mild or modest aortic dilatation is common in repaired TOF
patients.71
Finally, LGE allows the detection of fibrosis that has been reported
to be related to the risk of ventricular tachycardia and sudden cardiac
death in this patients.72
Cardiac CT provides information on the extent of conduit calcification, postsurgical PA stenosis, coronary arteries, and lung parenchyma and has a specific role in the planning for percutaneous
pulmonary valve implantation to assess the degree of calcification
and the proximity of the coronary arteries.

12

S. Orwat et al.

of the superior limb of the systemic venous atrium (SVA). SVC, superior vena cava. Stenosis at the isthmus of the pulmonary venous atrium (PVA)
between its posterior (PVAp) and anterior segment (PVAa) revealed by TOE (C and D).

Figure 4: CT angiography of TGA after arterial switch and Lecompte-Maneuver. Normal dimension (A) and narrowing (B and C) of the left pulmonary arteries (LPA). Ao, aorta.

evaluation focuses on LV global and regional function, evaluation of

aortic root dimensions, as well as the presence and severity of AR.
Supravalvular pulmonary stenosis (PS) and PA branch stenosis may
be difficult to visualize and elevated RVP as indicated by tricuspid
regurgitation velocity is then an important indirect sign guiding
further evaluation.
CT is an additional adjunct that can be used for non-invasive
imaging of coronary arteries, including the ostia, and in the case of
suspicion of pulmonary branch stenosis as an alternative to CMR
(Figure 4).

In the case of inconclusive CT or CMR imaging of the coronary

arteries and the evaluation of RVOTO, cardiac catheterization is
indicated.1
The third type of operation for TGA is the Rastelli procedure, in
which the anatomical LV is connected through a VSD patch to the
aorta and the RV is connected with a valved conduit to the PA in
patients with a suitable VSD.80 Echocardiography is used among
others to assess the function of the conduit between RV and the pulmonary trunk, but Doppler gradients may be unreliable. Therefore,
RVP estimation from tricuspid regurgitation velocity is of particular

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Figure 3: Assessment of intra-atrial tunnels in TGA repaired by Mustard operation. Angiogram (A) and CMR (B) demonstrate narrowing (arrows)

13

Imaging of congenital heart disease in adults

importance for the assessment of conduit stenosis. For morphological assessment, CMR and CT are used.

Coarctation of the aorta

Univentricular heart and Fontan

circulation
The term univentricular heart encompasses a wide spectrum of conditions in which one dominant functional chamber and nearly always a
second hypoplastic accessory ventricle exists.88 For many years now,
the Fontan operation in various modifications has become the palliation procedure of choice with direct connection of the superior and
inferior vena cava to the pulmonary arteries.
With echocardiography, the morphology of the dominant
chamber and basic anatomy of the heart defect as well as the systolic
function of the dominant ventricle, the morphology and function of
the valves, as well as the presence of a restriction to pulmonary
blood flow can generally be assessed. However, in most of the
cases, complete evaluation including the venous and arterial
anatomy requires CMR and/or CT in addition.
In patients after Fontan palliation, CMR or CT is used to evaluate
the patency of cavo-pulmonary pathways (this may require simultaneous injection of contrast agent through the arm and leg veins) and in
combination with cardiac catheterization to assess the shunt fenestration flow89 (Figure 6).
CMR is also the method of choice for quantification of ventricular
function and relative distribution of blood flow in the left and right
lungs. Comprehensive coverage using a contiguous stack of transaxial
cines is recommended, this generally being suitable for the identification of any intra-atrial thrombus or stenosis of the cavo-pulmonary
connections19 and helps to evaluate hepatic congestion. Myocardial
fibrosis as identified by LGE is a common finding and is associated
with systolic dysfunction and arrhythmias.90
Frequently, cardiac catheterization is necessary for treatment decision because of missing information on hemodynamic significance
of stenosis, PAP, pulmonary vascular resistance, and collateral
vessels.

Figure 5: (A) Coarctation of the aorta. Continuous-wave Doppler from a suprasternal approach with diastolic prolongation of forward flow (diastolic tail), (B) 3-D reconstruction based on CT angiography shows a severe stenosis (arrow) with collaterals.

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Aortic coarctation (CoA) is characterized by a narrowing of the aorta

close to the area where the arterial duct inserts (distal to the origin of
the left subclavian artery). The condition is considered to be part of a
generalized arteriopathy and is commonly associated with other
lesions of the aorta, such as bicuspid aortic valve, and hypoplasia of
the arch. The majority of adult patients will have undergone surgical
repair in childhood and some of them will suffer from aortic complications such as restenosis, aneurysm formation of the repair site, and
dissection.81,82
With echocardiography CoA or re-coarctation can be sometimes
visualized from a suprasternal view. While high systolic velocities are
commonly found on continuous-wave Doppler, this does not represent a reliable sign of significant obstruction. High Doppler velocities
that continue throughout the cardiac cycle, resulting in a diastolic
run-off or diastolic tail phenomenon indicate significant coarctation.83,84 (Figure 5)
Echo is not sufficient for the quantification and morphologic characterization of coarctation (and lacks the ability to reliably detect aneurysm formation) as well as re-coarctation, thus requiring additional
diagnostic modalities such as CMR and CT, which are the preferred
non-invasive techniques to evaluate the anatomy of the entire
aorta.85 If possible all patient should have at least one assessment
of the aortic anatomypreferably using CMRin adulthood followed by subsequent periodic assessments with intervals depending
on the individual pathologic findings. Both methods directly detect
residual stenosis, restenosis/re-coarctation, aneurysms or significance of collateral vessels, and CMR flow measurements allow for
the detection of diastolic prolongation of forward flowa concept
similar to the diastolic run-off on echo.86 CT has the advantage of
better resolution whereas CMR is superior by providing collateral
flow measurements.87
Nevertheless, echocardiography remains essential in this patient
group to assess associated lesions such bicuspid valve etc. and to
assess the response of the LV (hypertrophy and function) to

increased afterload due to hypertension with and without

re-coarctation. CT is particularly helpful in evaluation of patient
post-coarctation stenting. CMR multidirectional, 3-D cine velocity
acquisitions may help to detect altered patterns of aortic flow,
although the clinical significance is still unknown.38

14

S. Orwat et al.

Figure 6: (A) Balanced-SSFP cine image of Fontan pathways in a patient with a lateral tunnel (LT) and a fenestration closed by an occluder (arrow).
(B) CMR 3-D pathline visualization of blood flow in the lateral Fontan tunnel, left and right pulmonary arteries (LPA and RPA).

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Specific considerations in selected

lesions frequently encountered
undiagnosed in adult life
Like most paediatric patients with CHD, adults with previously undiagnosed anomalies benefit from a sequential, segmental approach
for the initial evaluation.2

Atrial septal defect

Atrial septal defect (ASD) is the most common CHD in adults and is
often undiagnosed until adulthood, due to a lack of early symptoms
and subtlety of physical findings. Depending on the location several
types of ASDs can be distinguished and several associated lesions
may exist. The secundum ASD is by far the most common type of
ASDs, which is localized centrally in the intra-atrial septum. In the
presence of unexplained RV volume overload, shunting at the atrial
level should always be suspected. Echocardiography remains the
key diagnostic technique, providing information of RV volume overload, size, and morphology of defect and exclusion of additional
defects. Direct defect visualization should be attempted by TTE
(short axis and subcostal view) and should be confirmed with
Doppler echocardiography. In case of poor visualization by TTE
(especially in the setting of sinus venosus defects) or uncertain diagnosis TOE should be employed (Figure 7). TOE also allows the visualization of pulmonary venous return and can be used to rule out
anomalous pulmonary venous connection. Three-dimensional TOE
has been proposed for appreciating the defects shape prior to and
during catheter interventional closure.91,92 In addition, good correlation between 3-D TOE measurements and balloon stretched ASD
diameters has been reported.93 Nevertheless, 2-D TOE remainsmore important for rim assessment and balloon measurement is
indispensable in clinical practice.
CMR can serve as an excellent supplement particularly for the
assessment of RV volume overload in borderline cases and the
confirmation of normal pulmonary venous connection.

Figure 7: (A) TOE of a superior sinus venous defect (arrow)

located at the entry of the superior vena cava (SVC) into the right
atrium. LA, left atrium; RA, right atrium.

Congenitally corrected transposition of

the great arteries
Congenitally corrected transposition of the great arteries (ccTGA) is
a rare condition characterized by atrioventricular (AV) and
ventricular-arterial discordance. As a consequence, the morphological RV supports the systemic circulation. Associated lesions are
common (e.g. VSD and PS); however, in isolated ccTGA patients
are often asymptomatic until adulthood and may remain undiagnosed
for a long time.
Deterioration of systemic ventricular function and systemic AV
valve regurgitation (tricuspid insufficiency) are well-known late
complications.62,94,95
Echocardiography is the mainstay of diagnosis, demonstrates the
double discordance, and helps to identify associated anomalies, particularly systemic AV valve abnormalities (Ebstein-like or dysplastic)
and regurgitation. (Figure 8)

Imaging of congenital heart disease in adults

15

Figure 8: CMR short axis (A) and four-chamber view (B) in a patient with congenitally corrected transposition of the great arteries demonstrating
AV discordance. The systemic AV (tricuspid) valve (TV) is guarding the systemic right (subaortic) ventricle (RV). Echocardiographic apical fourchamber view demonstrating severe systemic AV regurgitation (C).

Summary
Non-invasive imaging modalities are widely used to assess ACHD
patients, and the need for invasive assessment has been significantly
reduced in the last years. The assessment of ACHD has to involve
a variety of imaging modalities that should be used in a complementary fashion. Echocardiography remains the workhorse of imaging
routinely used in all patients but in complex disease and after
surgery echocardiography is frequently insufficient. CMR is particularly useful in this setting and allows reproducible and accurate quantification of ventricular function and comprehensive assessment of
cardiac anatomy, aorta, pulmonary arteries, and venous return including complex flow measurements. CT may be used when CMR
is contraindicated, when superior spatial resolution is required or
when metallic artefacts limit CMR imaging.
The challenge remains to appreciate the different advantages and
disadvantages of cardiovascular imaging modalities and to determine
the most appropriate and cost-effective diagnostic pathways for
individual ACHD patients.
Conflict of interest: none declared.

Funding
None.

References
1. Baumgartner H, Bonhoeffer P, De Groot NMS, de Haan F, Deanfield JE, Galie N
et al. ESC Guidelines for the management of grown-up congenital heart disease
(new version 2010). Eur Heart J 2010;31:2915 57.
2. Anderson RH, Becker AE, Freedom RM, Macartney FJ, Quero-Jimenez M,
Shinebourne EA et al. Sequential segmental analysis of congenital heart disease.
Pediatr Cardiol 1984;5:281 7.

3. Sugeng L, Mor-Avi V, Weinert L, Niel J, Ebner C, Steringer-Mascherbauer R et al.

Multimodality comparison of quantitative volumetric analysis of the right ventricle.
JACC Cardiovasc Imaging 2010;3:108.
4. Ho SY, Nihoyannopoulos P. Anatomy, echocardiography, and normal right ventricular dimensions. Heart 2006;92(Suppl. 1):12 13.
5. Haddad F, Doyle R, Murphy DJ, Hunt SA. Right ventricular function in cardiovascular disease, part II: pathophysiology, clinical importance, and management of right
ventricular failure. Circulation 2008;117:1717 31.
6. Khattab K, Schmidheiny P, Wustmann K, Wahl A, Seiler C, Schwerzmann M. Echocardiogram versus cardiac magnetic resonance imaging for assessing systolic function of subaortic right ventricle in adults with complete transposition of great
arteries and previous atrial switch operation. Am J Cardiol 2013;111:908 13.
7. Bleich S, Nanda NC, Hage FG. The incremental value of three-dimensional transthoracic echocardiography in adult congenital heart disease. Echocardiography
2013;30:483 94.
8. Vettukattil JJ. Three dimensional echocardiography in congenital heart disease.
Heart 2012;98:79 88.
9. van der Zwaan HB, Helbing WA, McGhie JS, Geleijnse ML, Luijnenburg SE,
Roos-Hesselink JW et al. Clinical value of real-time three-dimensional echocardiography for right ventricular quantification in congenital heart disease: validation with
cardiac magnetic resonance imaging. J Am Soc Echocardiogr 2010;23:13440.
10. Friedberg MK, Mertens L. Deformation imaging in selected congenital heart
disease: is it evolving to clinical use? J Am Soc Echocardiogr 2012;25:919 31.
11. Diller G-P, Kempny A, Liodakis E, Alonso-Gonzalez R, Inuzuka R, Uebing A et al.
Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of Fallot. Circulation 2012;125:
2440 6.
12. Diller G-P, Radojevic J, Kempny A, Alonso-Gonzalez R, Emmanouil L, Orwat S et al.
Systemic right ventricular longitudinal strain is reduced in adults with transposition
of the great arteries, relates to subpulmonary ventricular function, and predicts
adverse clinical outcome. Am Heart J 2012;163:85966.
13. Biaggi P, Carasso S, Garceau P, Greutmann M, Gruner C, Tsang W et al. Comparison
of two different speckle tracking software systems: does the method matter? Echocardiography 2011;28:539 47.
14. Sun JP, Lee AP-W, Wu C, Lam Y-Y, Hung M-J, Chen L et al. Quantification of left
ventricular regional myocardial function using two-dimensional speckle tracking
echocardiography in healthy volunteersa multi-center study. In J Cardiol 2013;
167:495 501.
15. Carasso S, Biaggi P, Rakowski H, Mutlak D, Lessick J, Aronson D et al. Velocity
vector imaging: standard tissue-tracking results acquired in normalsthe VVISTRAIN study. J Am Soc Echocardiogr 2012;25:543 52.
16. Marwick TH, Leano RL, Brown J, Sun JP, Hoffmann R, Lysyansky P et al. Myocardial
strain measurement with 2-dimensional speckle-tracking echocardiography: definition of normal range. JACC Cardiovasc Imaging 2009;2:80 4.
17. Nelson MR, Hurst RT, Raslan SF, Cha S, Wilansky S, Lester SJ. Echocardiographic
measures of myocardial deformation by speckle-tracking technologies: the need
for standardization? J Am Soc Echocardiogr 2012;25:1189 94.
18. Kramer CM, Barkhausen J, Flamm SD, Kim RJ, Nagel E, Society for Cardiovascular
Magnetic Resonance Board of Trustees Task Force on Standardized Protocols.
Standardized cardiovascular magnetic resonance imaging (CMR) protocols,
society for cardiovascular magnetic resonance: board of trustees task force on
standardized protocols. J Cardiovasc Magn Reson 2008;10:35.

Downloaded from by guest on January 31, 2015

CMR assessment of RV size and function is challenging due to the

complex shape and the multiple thick trabeculations of the systemic
right ventricle, and the use of a consistent and reproducible delineation of the cavity is important.96 CT helps to evaluate the complex
mirror-image distribution of coronary arteries. In most of the
cases, the morphologic right coronary artery arises from the left posterior sinus and gives rise to a circumflex artery, and the morphologic
left main coronary artery arises from the right anterior sinus.97

16

41. Broberg CS, Chugh SS, Conklin C, Sahn DJ, Jerosch-Herold M. Quantification of
diffuse myocardial fibrosis and its association with myocardial dysfunction in congenital heart disease. Circ Cardiovasc Imaging 2010;3:727 34.
42. Goo HW. Haemodynamic findings on cardiac CT in children with congenital heart
disease. Pediatr Radiol 2011;41:250 61.
43. Cook SC, Raman SV. Multidetector computed tomography in the adolescent and
young adult with congenital heart disease. J Cardiovasc Comput Tomogr 2008;2:
36 49.
44. Ghoshhajra BB, Sidhu MS, El-Sherief A, Rojas C, Yeh DD, Engel L-C et al. Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings. Congen Heart Dis 2012;7:516 25.
45. Flohr TG, Klotz E, Allmendinger T, Raupach R, Bruder H, Schmidt B. Pushing the
envelope. J Thorac Imaging 2010;25:10011.
46. American College of Cardiology Foundation Task Force on Expert Consensus
Documents, Mark DB, Berman DS, Budoff MJ, Carr JJ, Gerber TC et al. ACCF/
ACR/AHA/NASCI/SAIP/SCAI/SCCT 2010 expert consensus document on coronary computed tomographic angiography: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. J Am Coll Cardiol
2010;55:2663 99.
47. van der Wall EE. Crown years for non-invasive cardiovascular imaging (Part IV): 30
years of cardiac computed tomography. Neth Heart J 2013:1 4.
48. Dimopoulos K, Giannakoulas G, Bendayan I, Liodakis E, Petraco R, Diller G-P et al.
Cardiothoracic ratio from postero-anterior chest radiographs: a simple, reproducible and independent marker of disease severity and outcome in adults with congenital heart disease. Int J Cardiol 2013;166:4537.
49. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM et al. Long-term
outcome in patients undergoing surgical repair of tetralogy of Fallot. New Engl J Med
1993;329:593 9.
50. Lindberg HL, Saatvedt K, Seem E, Hoel T, Birkeland S. Single-center 50 years experience with surgical management of tetralogy of Fallot. Eur J Cardiothorac Surg
2011;40:538 42.
51. Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol 2002;40:1675 80.
52. Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ. Factors associated with
impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated
by magnetic resonance imaging. J Am Coll Cardiol 2004;43:1068 74.
53. Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JCC et al. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow
aneurysms or akinesia and adverse right-to-left ventricular interaction. J Am Coll
Cardiol 2002;40:2044 52.
54. Brown DW, McElhinney DB, Araoz PA, Zahn EM, Vincent JA, Cheatham JP et al.
Reliability and accuracy of echocardiographic right heart evaluation in the U.S.
Melody Valve Investigational Trial. J Am Soc Echocardiogr 2012;25:383 4.
55. Greutmann M, Tobler D, Biaggi P, Mah ML, Crean A, Oechslin EN et al. Echocardiography for assessment of right ventricular volumes revisited: a cardiac magnetic
resonance comparison study in adults with repaired tetralogy of Fallot. J Am Soc
Echocardiogr 2010;23:905 11.
56. Therrien J, Provost Y, Merchant N, Williams W, Colman J, Webb G. Optimal timing
for pulmonary valve replacement in adults after tetralogy of Fallot repair. Am J
Cardiol 2005;95:779 82.
57. Buechel ERV, Dave HH, Kellenberger CJ, Dodge-Khatami A, Pretre R, Berger F et al.
Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance. Eur Heart J 2005;26:2721 7.
58. Cullen S, Shore D, Redington A. Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot. Restrictive physiology predicts slow postoperative recovery. Circulation 1995;91:1782 9.
59. van den Berg J, Wielopolski PA, Meijboom FJ, Witsenburg M, Bogers AJJC,
Pattynama PMT et al. Diastolic function in repaired tetralogy of Fallot at rest and
during stress: assessment with MR imaging. Radiology 2007;243:212 9.
60. Apitz C, Latus H, Binder W, Uebing A, Seeger A, Bretschneider C et al. Impact of
restrictive physiology on intrinsic diastolic right ventricular function and lusitropy
in children and adolescents after repair of tetralogy of Fallot. Heart 2010;96:
1837 41.
61. Kempny A, Diller G-P, Orwat S, Kaleschke G, Kerckhoff G, Bunck AC et al. Right
ventricular-left ventricular interaction in adults with Tetralogy of Fallot: A combined cardiac magnetic resonance and echocardiographic speckle tracking study.
Int J Cardiol 2012;154:259 64.
62. Scherptong RWC, Mollema SA, Blom NA, Kroft LJM, Roos A, Vliegen HW et al.
Right ventricular peak systolic longitudinal strain is a sensitive marker for right ventricular deterioration in adult patients with tetralogy of Fallot. Int J Cardiovasc
Imaging 2009;25:66976.

Downloaded from by guest on January 31, 2015

19. Kilner PJ, Geva T, Kaemmerer H, Trindade PT, Schwitter J, Webb GD. Recommendations for cardiovascular magnetic resonance in adults with congenital heart
disease from the respective working groups of the European Society of Cardiology.
Eur Heart J 2010;31:794805.
20. Grothues F, Moon JC, Bellenger NG, Smith GS, Klein HU, Pennell DJ. Interstudy
reproducibility of right ventricular volumes, function, and mass with cardiovascular
magnetic resonance. Am Heart J 2004;147:218 23.
21. Pennell DJ, Sechtem UP, Higgins CB, Manning WJ, Pohost GM, Rademakers FE et al.
Clinical indications for cardiovascular magnetic resonance (CMR): Consensus
Panel report. Eur Heart J 2004;25:1940 65.
22. Maceira AM, Prasad SK, Khan M, Pennell DJ. Reference right ventricular systolic and
diastolic function normalized to age, gender and body surface area from
steady-state free precession cardiovascular magnetic resonance. Eur Heart J
2006;27:2879 88.
23. Bonello B, Kilner PJ. Review of the role of cardiovascular magnetic resonance in
congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc
Dis 2012;105:1 9.
24. Ibrahim E-SH. Myocardial tagging by cardiovascular magnetic resonance: evolution
of techniquespulse sequences, analysis algorithms, and applications. J Cardiovasc
Magn Reson 2011;13:36.
25. Maret E, Todt T, Brudin L, Nylander E, Swahn E, Ohlsson JL et al. Functional measurements based on feature tracking of cine magnetic resonance images identify left
ventricular segments with myocardial scar. Cardiovasc Ultrasound 2009;7:53.
26. Hor KN, Gottliebson WM, Carson C, Wash E, Cnota J, Fleck R et al. Comparison of
magnetic resonance feature tracking for strain calculation with harmonic phase
imaging analysis. JACC Cardiovasc Imaging 2010;3:144 51.
27. Hor KN, Baumann R, Pedrizzetti G, Tonti G, Gottliebson WM, Taylor M et al. Magnetic resonance derived myocardial strain assessment using feature tracking. J Vis
Exp 2011:e2356. doi:10.3791/2356.
28. Kempny A, Fernandez-Jimenez R, Orwat S, Schuler P, Bunck AC, Maintz D et al.
Quantification of biventricular myocardial function using cardiac magnetic resonance feature tracking, endocardial border delineation and echocardiographic
speckle tracking in patients with repaired tetralogy of Fallot and healthy controls.
J Cardiovasc Magn Reson 2012;14:32.
29. Kutty S, Rangamani S, Venkataraman J, Li L, Schuster A, Fletcher SE et al. Reduced
global longitudinal and radial strain with normal left ventricular ejection fraction late
after effective repair of aortic coarctation: a CMR feature tracking study. Int J Cardiovasc Imaging 2013;29:141 50.
30. Goldberg A, Jha S. Phase-contrast MRI and applications in congenital heart disease.
Clin Radiol 2012;67:399 410.
31. Joint Task Force on the Management of Valvular Heart Disease of the European
Society of Cardiology (ESC), European Association for Cardio-Thoracic Surgery
(EACTS), Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Baron-Esquivias G,
Baumgartner H et al. Guidelines on the management of valvular heart disease
(version 2012). Eur Heart J 2012;109:2451 96.
32. Roest AAW, de Roos A. Imaging of patients with congenital heart disease. Nat Rev
Cardiol 2012;9:101 15.
33. Chatzimavroudis GP, Walker PG, Oshinski JN, Franch RH, Pettigrew RI,
Yoganathan AP. Slice location dependence of aortic regurgitation measurements
with MR phase velocity mapping. Magn Reson Med 1997;37:54551.
34. Bogren HG, Klipstein RH, Firmin DN, Mohiaddin RH, Underwood SR, Rees RS et al.
Quantitation of antegrade and retrograde blood flow in the human aorta by magnetic resonance velocity mapping. Am Heart J 1989;117:1214 22.
35. Hundley WG, Li HF, Lange RA, Pfeifer DP, Meshack BM, Willard JE et al. Assessment
of left-to-right intracardiac shunting by velocity-encoded, phase-difference magnetic resonance imaging. A comparison with oximetric and indicator dilution techniques. Circulation 1995;91:2955 60.
36. Beerbaum P, Korperich H, Barth P, Esdorn H, Gieseke J, Meyer H. Noninvasive
quantification of left-to-right shunt in pediatric patients: phase-contrast cine magnetic resonance imaging compared with invasive oximetry. Circulation 2001;103:
2476 82.
37. Hope MD, Meadows AK, Hope TA, Ordovas KG, Saloner D, Reddy GP et al. Clinical evaluation of aortic coarctation with 4D flow MR imaging. J Magn Reson Imaging
2010;31:711 8.
38. Markl M, Kilner PJ, Ebbers T. Comprehensive 4D velocity mapping of the heart and
great vessels by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2011;
13:7.
39. Mooij CF, de Wit CJ, Graham DA, Powell AJ, Geva T. Reproducibility of MRI measurements of right ventricular size and function in patients with normal and dilated
ventricles. J Magn Reson Imaging 2008;28:67 73.
40. Coelho-Filho OR, Mongeon F-P, Mitchell R, Moreno H, Nadruz W, Kwong R et al.
The role of transcytolemmal water exchange in magnetic resonance measurements of diffuse myocardial fibrosis in hypertensive heart disease. Circ Cardiovasc
Imaging 2013;6:134 41.

S. Orwat et al.

17

Imaging of congenital heart disease in adults

83.

84.

85.

86.

87.
88.
89.

90.

91.

92.
93.

94.

95.

96.

97.

98.

99.

100.

101.

102.

absence of restenosis and prosthetic material. J Thorac Cardiovasc Surg 2007;134:

738 45.
Tan J-L, Babu-Narayan SV, Henein MY, Mullen M, Li W. Doppler echocardiographic
profile and indexes in the evaluation of aortic coarctation in patients before and
after stenting. J Am Coll Cardiol 2005;46:1045 53.
Carvalho JS, Redington AN, Shinebourne EA, Rigby ML, Gibson D. Continuous
wave Doppler echocardiography and coarctation of the aorta: gradients and
flow patterns in the assessment of severity. Br Heart J 1990;64:133 7.
Parks WJ, Ngo TD, Plauth WH, Bank ER, Sheppard SK, Pettigrew RI et al. Incidence
of aneurysm formation after Dacron patch aortoplasty repair for coarctation of the
aorta: long-term results and assessment utilizing magnetic resonance angiography
with three-dimensional surface rendering. J Am Coll Cardiol 1995;26:266 71.
Therrien J, Thorne SA, Wright A, Kilner PJ, Somerville J. Repaired coarctation: a
cost-effective approach to identify complications in adults. J Am Coll Cardiol
2000;35:997 1002.
Rosenthal E, Bell A. Optimal imaging after coarctation stenting. Heart 2010;96:
1169 71.
Khairy P, Poirier N, Mercier L-A. Univentricular heart. Circulation 2007;115:
800 12.
Wagner M, Nguyen K-L, Khan S, Mirsadraee S, Satou GM, Aboulhosn J et al.
Contrast-enhanced MR angiography of cavopulmonary connections in adult
patients with congenital heart disease. Am J Roentgenol 2012;199:W565 74.
Rathod RH, Prakash A, Powell AJ, Geva T. Myocardial fibrosis identified by cardiac
magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll
Cardiol 2010;55:1721 8.
Saric M, Perk G, Purgess JR, Kronzon I. Imaging atrial septal defects by real-time
three-dimensional transesophageal echocardiography: step-by-step approach.
J Am Soc Echocardiogr 2010;23:1128 35.
Shirali GS. Three-dimensional echocardiography in congenital heart disease. Echocardiography 2012;29:242 8.
Seo J-S, Song J-M, Kim Y-H, Park D-W, Lee S-W, Kim W-J et al. Effect of atrial septal
defect shape evaluated using three-dimensional transesophageal echocardiography on size measurements for percutaneous closure. J Am Soc Echocardiogr
2012;25:1031 40.
Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM. Progressive tricuspid
valve disease in patients with congenitally corrected transposition of the great arteries. Circulation 1998;98:997 1005.
Mongeon F-P, Connolly HM, Dearani JA, Li Z, Warnes CA. Congenitally corrected
transposition of the great arteries ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function. J Am Coll
Cardiol 2011;57:2008 17.
Winter MM, Bernink FJ, Groenink M, Bouma BJ, van Dijk AP, Helbing WA et al.
Evaluating the systemic right ventricle by CMR: the importance of consistent and
reproducible delineation of the cavity. J Cardiovasc Magn Reson 2008;10:40.
Kantarci M, Koplay M, Bayraktutan U, Gundogdu F, Ceviz N. Congenitally corrected transposition of the great arteries: MDCT angiography findings and interpretation of complex coronary anatomy. Int J Cardiovasc Imaging 2006;23:405 10.
Dave HH, Buechel ERV, Dodge-Khatami A, Kadner A, Rousson V, Bauersfeld U et al.
Early insertion of a pulmonary valve for chronic regurgitation helps restoration of
ventricular dimensions. Ann Thorac Surg 2005;80:1615 20.
Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk APJ,
Spijkerboer AM et al. Preoperative thresholds for pulmonary valve replacement
in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 2007;116:545 51.
Frigiola A, Tsang V, Bull C, Coats L, Khambadkone S, Derrick G et al. Biventricular
response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation 2008;118:S182 90.
Geva T, Gauvreau K, Powell AJ, Cecchin F, Rhodes J, Geva J et al. Randomized trial of
pulmonary valve replacement with and without right ventricular remodeling
surgery. Circulation 2010;122:S2018.
Lee C, Kim YM, Lee C-H, Kwak JG, Park C-S, Song JY et al. Outcomes of pulmonary
valve replacement in 170 patients with chronic pulmonary regurgitation after relief
of right ventricular outflow tract obstruction: implications for optimal timing of
pulmonary valve replacement. J Am Coll Cardiol 2012;60:1005 14.

Downloaded from by guest on January 31, 2015

63. Friedberg MK, Fernandes FP, Roche SL, Grosse-Wortmann L, Manlhiot C,

Fackoury C et al. Impaired right and left ventricular diastolic myocardial mechanics
and filling in asymptomatic children and adolescents after repair of tetralogy of
Fallot. Eur Heart J Cardiovasc Imaging 2012;13:90513.
64. Mercer-Rosa L, Yang W, Kutty S, Rychik J, Fogel M, Goldmuntz E. Quantifying pulmonary regurgitation and right ventricular function in surgically repaired tetralogy
of Fallot: a comparative analysis of echocardiography and magnetic resonance
imaging. Circ Cardiovasc Imaging 2012;5:63743.
65. Helbing WA, De Roos A. Optimal imaging in assessment of right ventricular function in tetralogy of Fallot with pulmonary regurgitation. Am J Cardiol 1998;82:
15612.
66. Rebergen S, Chin J, Ottenkamp J, Van Der Wall E, De Roos A. Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping. Circulation 1993;88:2257.
67. Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision
support. J Cardiovasc Magn Reson 2011;13:9.
68. Oosterhof T, Mulder BJM, Vliegen HW, de Roos A. Cardiovascular magnetic resonance in the follow-up of patients with corrected tetralogy of Fallot: a review.
Am Heart J 2006;151:26572.
69. Clarke CJ, Gurka MJ, Norton PT, Kramer CM, Hoyer AW. Assessment of the accuracy and reproducibility of RV volume measurements by CMR in congenital heart
disease. JACC Cardiovasc Imaging 2012;5:28 37.
70. Wald RM, Redington AN, Pereira A, Provost YL, Paul NS, Oechslin EN et al. Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot
repair: should we be measuring regurgitant fraction or regurgitant volume? Eur
Heart J 2009;30:356 61.
71. Kay WA, Cook SC, Daniels CJ. Evaluation by MRA of aortic dilation late after repair
of tetralogy of Fallot. International journal of cardiology. Elsevier Ireland Ltd 2012;14:
16.
72. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA et al. Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with
repaired tetralogy of Fallot and its relationship to adverse markers of clinical
outcome. Circulation 2006;113:405 13.
73. Khairy P, Landzberg MJ, Lambert J, ODonnell CP. Long-term outcomes after the
atrial switch for surgical correction of transposition: a meta-analysis comparing
the Mustard and Senning procedures. Cardiol Young 2004;14:284 92.
74. Lindqvist P, Calcutteea A, Henein M. Echocardiography in the assessment of right
heart function. Eur J Echocardiogr 2008;9:225 34.
75. Becker M, Humpel C, Ocklenburg C, Muehler E, Schroeder J, Eickholt C et al. The
right ventricular response to high afterload: comparison between healthy persons
and patients with transposition of the great arteries: a 2D strain study. Echocardiography 2010;27:1256 62.
76. Iriart X, Horovitz A, van Geldorp IE, Barnetche T, Lederlin M, De Guillebon M et al.
The role of echocardiography in the assessment of right ventricular systolic function in patients with transposition of the great arteries and atrial redirection. Arch
Cardiovasc Dis 2012;105:432 41.
77. Dorfman AL, Geva T. Magnetic resonance imaging evaluation of congenital heart
disease: conotruncal anomalies. J Cardiovasc Magn Reson 2006;8:645 59.
78. Losay J, Touchot A, Serraf A, Litvinova A, Lambert V, Piot JD et al. Late outcome
after arterial switch operation for transposition of the great arteries. Circulation
2001;104(12 Suppl. 1):I121 6.
79. Schwartz ML, Gauvreau K, del Nido P, Mayer JE, Colan SD. Long-term predictors of
aortic root dilation and aortic regurgitation after arterial switch operation. Circulation 2004;110(11 Suppl. 1):II128 32.
80. Rastelli GC, Wallace RB, Ongley PA. Complete repair of transposition of the great
arteries with pulmonary stenosis. A review and report of a case corrected by using a
new surgical technique. Circulation 1969;39:8395.
81. Himyr H, Christensen TD, Emmertsen K, Johnsen SP, Riis A, Hansen OK et al. Surgical repair of coarctation of the aorta: up to 40 years of follow-up. Eur J Cardiothorac
Surg 2006;30:9106.
82. Hager A, Kanz S, Kaemmerer H, Schreiber C, Hess J. Coarctation Long-term Assessment (COALA): significance of arterial hypertension in a cohort of 404 patients
up to 27 years after surgical repair of isolated coarctation of the aorta, even in the