imaging of congenital heart disease in adultsimaging of congenital heart disease in adults...

Imaging of congenital heart disease in adults Babu-Narayan, Giannakoulas, Valente, Li and Gatzoulis

1

Imaging of congenital heart disease in adults

Sonya V. Babu-Narayan MB BS, BSc, MRCP, PhD1,

George Giannakoulas MD2, Anne Marie Valente MD,

3 Wei Li, MD PhD,

1

*Michael A. Gatzoulis MD, FRCPH, PhD1

1NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, UK and National Heart and

Lung Institute, Imperial College, UK

2 Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki

3 Boston Children’s Hospital and Brigham and Women’s Hospital, Harvard Medical School, Boston,

Massachusetts, USA

Address for correspondence:

*Michael A. Gatzoulis, MD PhD FACC FESC

Professor of Cardiology, Congenital Heart Disease and Consultant Cardiologist,

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, and the

National Heart & Lung Institute, Imperial College, London, UK.

Correspondence: Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.

Tel.: +44 20 73518602 Fax: +44 20 73518629 E-mail: [email protected]

http://www.ncbi.nlm.nih.gov/pubmed?term=Gatzoulis%20MA%5BAuthor%5D&cauthor=true&cauthor_uid=22465348

https://webmail.rbht.nhs.uk/OWA/UrlBlockedError.aspx


2

INTRODUCTION

The number of adults with congenital heart disease continues to increase due to significant advances

in early diagnosis and management.1 However, residual or postoperative right and left sided anatomic and

haemodynamic abnormalities are common. Cardiovascular imaging is essential in the long-term care of

adult congenital heart disease (ACHD). Periodic surveillance imaging is important for detecting

haemodynamic change, as symptoms may be late.2 With age, comorbidity including acquired heart disease

also comes into play.3 The choice and frequency of imaging modality is determined by lesion specific

patient characteristics, strengths and weaknesses of imaging modality and institutional resources and

expertise. A multimodality imaging approach is often required to obtain all the necessary information.

USE OF DIFFERENT IMAGING MODALITIES IN LIFELONG FOLLOW UP

Echocardiography

Echocardiography remains the workhorse and first line in imaging for assessment of anatomy and

physiology. It is widely available, portable, has high temporal resolution and is free from ionising radiation

exposure. Small, mobile intracardiac structures such as valves and vegetations and small intracardiac shunts

are well shown (Figure 1). Doppler echocardiography is usually the superior method for noninvasive

haemodynamic assessment of right ventricular (RV) and pulmonary artery (PA) pressure gradients and

valvular pathology and function in ACHD. To assess RV systolic function percentage fractional area change

(FAC) and tricuspid annular plane systolic excursion (TAPSE) from a four-chamber view are widely used.

TAPSE represents longitudinal contractile function of the RV, is reproducible and easy to measure though it

may be influenced by tricuspid regurgitation, abnormal ventricular geometry and recent surgical procedures.

Moreover, TAPSE has prognostic value, for example, in Eisenmenger syndrome,4 (Figure 2). Tissue

Doppler imaging of myocardial velocities and speckle tracking for myocardial deformation have both been

applied in ACHD for regional and global ventricular myocardial deformation assessment (Figure 3), but

their clinical application to the RV in ACHD remains to be elucidated. Transthoracic echocardiography


3

(TTE) acoustic windows may become suboptimal as patients age or have chest wall deformity from prior

surgery. Obtaining 3D echocardiography images that include all of the RV in ACHD is not always possible

and RV volumes may be underestimated. Transoesophageal echocardiography (TOE) is useful in selected

cases to guide interventional procedures (Figure 4) or further evaluate valvular anatomy.

CMR and CT

Cardiovascular magnetic resonance (CMR) allows unlimited access to imaging the heart and thoracic

cavity unrestricted by rib spaces, does not involve ionising radiation, and is therefore ideally suited and

recommended for long-term ACHD follow-up. 5 In addition to addressing many questions that TTE can

answer, CMR is the reference standard for accurate and reproducible quantification of right and left

ventricular volumes, mass and function.5 Other strengths include assessment of degree of valvar

dysfunction, especially pulmonary regurgitation, multilevel outflow tract obstruction, shunt quantification

(from pulmonary and systemic flows), differential branch pulmonary artery (PA) flow, and non-invasive

tissue characterisation. Advances in CMR allow for acquisition and reconstruction of comprehensive data

sets in any plane for subsequent analysis of anatomy, function and flow. In patients scheduled for

reoperation CMR (or CT) provides information to assess the relationship between vascular structures, the

heart and the sternum. Limitations include availability, higher cost, artefacts from stainless steel implants,

and relative contraindication in patients with pacemakers or implantable defibrillators (ICD). Magnetic

resonance-compatible devices are increasingly used and are desirable for ACHD patients; selected patients

with in situ conventional devices may safely undergo CMR with appropriate local protocol.6 However,

predicting the impact of artifact noise on image quality is difficult. Siting the device on the opposite side of

the chest from the heart may be preferable in ACHD patients.

Multi-detector ECG-gated cardiac computed tomography (CT) has excellent 3D spatial resolution

and allows for detailed evaluation of small blood vessels such as coronary arteries (Figure 5), pulmonary

veins, (Figure 6), collaterals, arteriovenous malformations, distal PA branches and in situ pulmonary

thrombosis. Acquisition time is rapid (< 2 minutes) allowing patients who are unable to lie still or flat for


4

long to be imaged. CT may also have added value in younger patients, for instance to assess aberrant

coronary anatomy. Pulmonary parenchyma imaging is also provided which is highly relevant for patients

with pulmonary hypertension. Furthermore, CT complements assessment of mechanical heart valve

dysfunction, and allows 3D visualization of abcess formation in endocarditis. CT is an alternative to

selective coronary angiography in older patients referred for ACHD surgery. However, CT exposes the

patient to ionising radiation and iodinated contrast agents, and does not provide information on

haemodynamics, flow rate or velocity. CT can be used to acquire ventricular volumes and function but with

lower temporal resolution than CMR or echocardiography and at the expense of additional radiation

exposure. It tends to overestimate ventricular volumes and is clearly unattractive for serial measurements

because of radiation.7 Multidetector CT enables dose reduction algorithms and is routinely practiced in our

centre.

Chest-x ray, nuclear scintigraphy and cardiac catheterisation

Chest x-ray is simple, cheap and reproducible and provides diagnostic information; furthermore,

cardiothoracic ratio relates to functional class and predicts survival in ACHD8 (Figure 6). Nuclear

scintigraphy has been reserved for selected patients only, for example for myocardial stress perfusion

imaging or differential pulmonary blood flow quantification when CMR is not available. Diagnostic cardiac

catheterisation and angiography are less frequently performed in ACHD nowadays; they are reserved for

specific clinical indications such as calculation of pulmonary vascular resistance or where the diagnosis

remains uncertain after noninvasive imaging or for percutaneous interventions.

IMAGING GOALS IN SPECIFIC CONGENITAL HEART DISEASES

Selected lesions are discussed in subsections below and the goals of imaging are summarised in Table 1.

Left ventricular outflow tract obstruction and aortic coarctation

Echocardiography is used to assess left ventricular outflow tract obstruction for example due to

subaortic ridge, aortic valvar and supravalvar stenosis plus aortic coarctation or re-coarctation; Doppler


5

derived diastolic tail in the descending thoracic aorta and continuous abdominal aortic flow indicate

significant coarctation.9 Additional information, such as aortic valve morphology, presence of ventricular

septal defect or left ventricular hypertrophy is sought. Aortopathy involving the aortic root or proximal

ascending aorta should be sought on 2D echocardiography.10

Repaired coarctation patients require screening

for the complications of recoarctation or aneurysm formation (Figure 7). Echo Doppler may reveal

nonspecific high-flow velocities across the isthmus in patients after coarctation repair given the fact that

elasticity of the aortic wall is lost in this trajectory, causing flow velocities to increase even in the absence of

stenosis. CMR is the gold standard for assessing LV mass and can be useful to assess multilevel left

ventricular outflow tract obstruction, aortic valve morphology, calibre of the entire aorta, and collateral flow.

All adults with aortic coarctation should undergo cross-sectional imaging (usually CMR) at least once.11

CT

is more suited for assessing stent lumen and fracture.

Atrial septal defects

The diagnosis of an atrial septal defect (ASD) and/or anomalous pulmonary veins should be

considered in adults presenting with right ventricular dilation. TTE is used to assess size and location of the

defect (secundum and primum), (Figure 4) TOE is the gold standard for accurately assessing size, number,

rims, and relationships to important neighbouring structures to determine suitability for percutaneous device

closure. CT and CMR are especially useful for detecting associated anomalous pulmonary veins that insert

into the superior vena cava above the level of the azygous vein. Transthoracic echocardiography may be

suitable for sinus venosus ASD imaging, however TOE, CMR or CT are often needed to delineate the

frequent association of anomalous pulmonary venous return (Figure 8). CMR and CT give information on

the distance of the anomalous pulmonary vein from the cardiac mass, which in turn is important for planning

the surgical approach to pulmonary venous redirection.

Atrioventricular septal defect

Patients with atrioventricular septal (canal) defects have a common atrioventricular junction with a

spectrum of lesions with potential for shunting at atrial level (ostium primum defects), ventricular level or


6

both atrial and ventricular level. The most common adult complication for repaired patients is left

atrioventricular valvar regurgitation (of note this valve has abnormal tri-leaflet morphology). Imaging must

also assess for other potential complications including pulmonary hypertension and left ventricular outflow

tract obstruction. TTE is usually able to address the majority of concerns and TOE used to assess for

suitability for repair versus replacement of the left atrioventricular valve.

Repaired tetralogy of Fallot and right ventricular outflow tract obstruction

Patients with repaired tetralogy of Fallot (TOF) constitute one of the largest groups of ACHD

patients surviving into adulthood. Residual hemodynamic and electrophysiological abnormalities contribute

to increasing morbidity and mortality rates arising in adulthood.12

The surgical strategy for TOF repair,

particularly with regards to right ventricular outflow tract (RVOT) reconstruction, has evolved with time.13

Conduits from the RV to pulmonary artery are sometimes required for TOF repair, for example with

associated pulmonary atresia or anomalous coronary arteries. These may be difficult to assess with TTE

alone due to their anterior location. A multimodality imaging approach is often utilised.14

Pulmonary

regurgitation is common and is an important factor for the long-term outcome of these patients. Aortic root

dilation is also common in patients with repaired TOF in some patients associated with significant aortic

regurgitation.15, 16

. CMR is recommended for all patients and is especially helpful for multilevel RVOT

(including branch pulmonary artery) obstruction, pulmonary regurgitation and RV volumes.16

RVOT

regional wall motion abnormalities and aneurysms are common and contribute to RV systolic dysfunction

and adverse ventricular interactions.17

RVOT akinetic area length predicts the onset of sustained ventricular

arrhythmia. Pulmonary regurgitation can be assessed with echocardiography18

although the gold standard for

its quantification is CMR. Free pulmonary regurgitation, that is whereby forward and reverse flow jets are

comparable in size, and imaging shows little or no effective remaining valve tissue, typically occurs with

regurgitation fraction 30-40%. Quantification of right atrial size is helpful as large right atrial area has been

associated with sustained atrial tachyarrhythmias in these patients.19


7

RV volumes quantified by CMR are followed serially for progressive dilatation; it has been

suggested that elective pulmonary valve replacement should be considered before RV end-diastolic volume

indexed to body surface area reaches 150-160mL/m2.20

RV volumes can also be assessed to define the

degree of RV reverse remodelling following valve implantation (Figure 9). Echocardiography can identify

the presence of a restrictive right ventricular physiology with the presence of an antegrade “a” wave (end-

diastolic forward flow) in the RV outflow tract on pulse wave Doppler throughout the respiratory cycle

demonstrating a non-compliant RV which is unable to distend further with atrial systole.24

This again may be

relevant in interpreting pulmonary regurgitant fraction or volume21

and RV volumes from CMR for timing

of pulmonary valve replacement. Left ventricular dysfunction is present in >20% of adults with TOF22

and is

associated with increased mortality.23, 24

Focal RV fibrosis on late gadolinium enhancement CMR imaging is associated with adverse clinical

prognosticators in adults with repaired TOF; prospective studies are required.25, 26

The INDICATOR

prospective study of 873 repaired TOF patients showed that CMR derived RV and LV ejection fraction

predict sustained ventricular tachycardia and mortality. 23

Cross-sectional imaging prior to catheter-based pulmonary valve reinterventions can be informative

regarding the origins and proximal course of the coronary arteries in relation to the RVOT (Figure 10)

Furthermore, cardiac CT provides information on the extent of conduit calcification for stent deployment.

Prior to redo sternotomy, cross sectional imaging shows the proximity or even adherence of the anterior RV

wall and/or ascending aorta to the sternum, thus allowing for surgical planning and avoidance of injury.

Systemic right ventricle after atrial redirection for TGA or in the setting of congenitally corrected

transposition of the great arteries (ccTGA)

Many surviving adults with TGA would have had atrial switch surgery, (Mustard or Senning

operation). Systemic RV dysfunction is a determining factor for late morbidity and mortality.

Echocardiographic parameters (RV FAC, TAPSE) can be used for detecting changes in RV function. Total

isovolumic time and peak systolic strain measures have prognostic value. Systemic atrioventricular


8

(tricuspid) valve regurgitation usually reflects progressive RV dilatation and dysfunction and is most

sensitively assessed by echocardiography, as is the presence of pulmonary hypertension or baffle leaks

(better delineated with the use of contrast). Associated lesions such as pulmonary stenosis and ventricular

septal defect can also be assessed. Pulmonary hypertension can be difficult to diagnose when there is no

mitral regurgitation but equal LV and RV size on apical four-chamber view is suggestive of pulmonary

hypertension or significant baffle leak. Imaging of patients after a Mustard or Senning operation requires

assessment of all three baffled atrial flow pathways (superior/inferior caval, and pulmonary venous atrial)

for presence and degree of obstruction. Increased flow velocity >1.6m/s or continuous baffle flow are

suggestive of obstruction. CMR in addition to echocardiography is often helpful for assessment of baffle

patency.

ccTGA comprises atrioventricular and ventriculoarterial discordance (L-loop TGA/double discordance). The

spectrum of clinical presentation is wide and depends on associated lesions; presentation in adulthood is

possible. The tricuspid valve in ccTGA is often intrinsically abnormal (Ebstein type and may be

underdiagnosed) and results in tricuspid regurgitation; the latter may also be secondary to RV dysfunction

and annular dilatation. Echocardiography can be used to assess RV dysfunction and tricuspid regurgitation.

Diastolic dysfunction can be assessed by Doppler flow of RV filling pattern. Absent Doppler “A” wave in

the presence of sinus rhythm and clear p waves often indicate very high filling pressures. CMR allows gold

standard quantification of systemic RV ejection fraction. This may be used to enable clinical decision-

making with regards to tricuspid valve surgical replacement. CT may be of value in the setting of systemic

RV dysfunction with wide QRS when cardiac resynchronisation is contemplated to assess the coronary sinus

anatomy. Tissue characterisation by CMR has shown areas of late gadolinium enhancement consistent with

focal RV fibrosis.27, 28

(Figure 11)., We have recently shown that systemic RV late gadolinium enhancement

correlates with histological fibrosis, is associated with clinical disease progression and predicts outcomes.29

justifying its periodic use. In our practice, we consider patients with RV ejection fraction of < 35% and


9

extensive RV fibrosis for primary prevention for sudden cardiac death; implantable defibrillator is offered

on an individualized basis following multidisciplinary discussion.

Transposition of the great arteries (TGA) after arterial switch surgery

Arterial switch procedure, i.e. anatomic repair, involving switching the great vessels and

reimplanting the coronary arteries has been the treatment of choice for infants with TGA over the past 3

decades. Supravalvar pulmonary stenosis, neo-aortic root dilatation, aortic valve regurgitation, left

ventricular dysfunction, and coronary occlusion are relatively common complications. Echocardiography

with Doppler can assess aortic valve and LV function and right ventricular pressures, whereas the main and

branch pulmonary arteries are often difficult to image. CMR (or CT) can provide detailed information on

RVOT or branch pulmonary artery stenoses. CT is particularly suited to image the proximal coronary

arteries, reimplanted during repair. CMR evaluation of coronary origins is also excellent although CT is

superior in excluding coronary stenoses. In the case of symptoms, LV dysfunction or LV scar at CMR

assessment of viability with CMR stress perfusion and with exercise echocardiography should be performed.

Single ventricle, Fontan procedure

Many survivors to adulthood of “single ventricle” physiology have undergone the Fontan operation to re-

route systemic venous return to the pulmonary arteries. In the earlier era this was done with an anastomosis

from the right atrial appendage to the pulmonary arteries (atriopulmonary Fontan), whereas more recently,

total cavopulmonary connection (TCPC), either using an intraatrial/lateral tunnel or extracardiac conduit.

The superior vena cava is connected end to side to the top of the right PA, whereas the inferior vena cava is

channelled by a patch, flap, or conduit up one side of the right atrium to the pulmonary artery. Thrombus

may form in the dilated right atrium in the atriopulmonary Fontan due to sluggish flow (Figure 12), or in the

disconnected pulmonary trunk after TCPC. Failure of the Fontan type circulation ensues with obstruction of

Fontan pathways, ventricular and or valvular dysfunction. Restrictive ventricular septal defect in the setting

of ventricular arterial discordance is unfavourable, causing subaortic stenosis. Ventricular systolic and

diastolic dysfunction determine outcome; echocardiography can assess both. CMR can establish patency of


10

pathways, exclude thrombus, quantify the volume of the dominant/primary ventricle and its ejection

fraction, and has demonstrated areas of ventricular fibrosis more commonly in patients with documented

non-sustained ventricular tachycardia.30

SUMMARY

Imaging is fundamental to the lifelong care of ACHD patients. Echocardiography remains the first line

imaging for inpatient, outpatient, or perioperative care. Cross-sectional imaging with CMR or CT provides

complementary and invaluable information on cardiac and vascular anatomy and other intra-thoracic

structures. Furthermore, CMR provides quantification of cardiac function and vascular flow. Cardiac

catheterisation is, mostly reserved for assessment of pulmonary vascular resistance, ventricular end-diastolic

pressure and percutaneous interventions. There have been further advances in non-invasive imaging for

ACHD including the application of advanced echocardiographic techniques, faster automated CMR imaging

and radiation dose reduction in CT. As a result ACHD, a heterogeneous population, benefit from appropriate

application of multiple imaging modalities matched with tertiary ACHD expertise.

Funding sources:

Sonya V. Babu-Narayan is supported by an Intermediate Clinical Research Fellowship from the British

Heart Foundation (FS/11/38/28864). This project was supported by the National Institute for Health

Research (NIHR) Cardiovascular Biomedical Research Unit of Royal Brompton and Harefield National

Health Service (NHS) Foundation Trust and Imperial College London. This report is independent research

by the NIHR Biomedical Research Unit Funding Scheme. The views expressed in this publication are those

of the author(s) and not necessarily those of the NHS, the National Institute for Health Research or the

Department of Health.

Conflict of interest: None.


11

REFERENCES

1. Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of

congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130:749-756

2. Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, Johansson B, Bouzas

B, Mullen MJ, Poole-Wilson PA, Francis DP, Gatzoulis MA. Exercise intolerance in adult congenital

heart disease: Comparative severity, correlates, and prognostic implication. Circulation.

2005;112:828-835

3. Giannakoulas G, Dimopoulos K, Engel R, Goktekin O, Kucukdurmaz Z, Vatankulu MA, Bedard E,

Diller GP, Papaphylactou M, Francis DP, Di Mario C, Gatzoulis MA. Burden of coronary artery

disease in adults with congenital heart disease and its relation to congenital and traditional heart risk

factors. Am J Cardiol. 2009;103:1445-1450

4. Moceri P, Dimopoulos K, Liodakis E, Germanakis I, Kempny A, Diller GP, Swan L, Wort SJ,

Marino PS, Gatzoulis MA, Li W. Echocardiographic predictors of outcome in eisenmenger

syndrome. Circulation. 2012;126:1461-1468

5. 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;31:794-805

6. Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G, Breithardt OA, Cleland J,

Deharo JC, Delgado V, Elliott PM, Gorenek B, Israel CW, Leclercq C, Linde C, Mont L, Padeletti L,

Sutton R, Vardas PE, Guidelines ESCCfP, Zamorano JL, Achenbach S, Baumgartner H, Bax JJ,

Bueno H, Dean V, Deaton C, Erol C, Fagard R, Ferrari R, Hasdai D, Hoes AW, Kirchhof P, Knuuti

J, Kolh P, Lancellotti P, Linhart A, Nihoyannopoulos P, Piepoli MF, Ponikowski P, Sirnes PA,

Tamargo JL, Tendera M, Torbicki A, Wijns W, Windecker S, Document R, Kirchhof P, Blomstrom-

Lundqvist C, Badano LP, Aliyev F, Bansch D, Baumgartner H, Bsata W, Buser P, Charron P,

Daubert JC, Dobreanu D, Faerestrand S, Hasdai D, Hoes AW, Le Heuzey JY, Mavrakis H,

McDonagh T, Merino JL, Nawar MM, Nielsen JC, Pieske B, Poposka L, Ruschitzka F, Tendera M,

Van Gelder IC, Wilson CM. 2013 ESC guidelines on cardiac pacing and cardiac resynchronization

therapy: The task force on cardiac pacing and resynchronization therapy of the european society of

cardiology (ESC). Developed in collaboration with the european heart rhythm association (EHRA).

Eur Heart J. 2013;34:2281-2329

7. Hoffmann A, Engelfriet P, Mulder B. Radiation exposure during follow-up of adults with congenital

heart disease. Int J Cardiol. 2007;118:151-153

8. Dimopoulos K, Giannakoulas G, Bendayan I, Liodakis E, Petraco R, Diller GP, Piepoli MF, Swan L,

Mullen M, Best N, Poole-Wilson PA, Francis DP, Rubens MB, Gatzoulis MA. 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;166:453-457

9. Tan JL, 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;46:1045-1053


12

10. Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK, Lai WW, Geva T.

Recommendations for quantification 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. 2010;23:465-495; quiz 576-

467

11. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules

JW, Graham TP, Jr., Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh

EP, Webb GD. ACC/AHA 2008 guidelines for the management of adults with congenital heart

disease: A report of the american college of cardiology/american heart association task force on

practice guidelines (writing committee to develop guidelines on the management of adults with

congenital heart disease). Circulation. 2008;118:e714-833

12. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M,

Moller JH, Gillette PC, Webb GD, Redington AN. Risk factors for arrhythmia and sudden cardiac

death late after repair of tetralogy of Fallot: A multicentre study. Lancet. 2000;356:975-981

13. Ghez O, Dimopoulos K, Babu-Narayan SV, Gatzoulis M. Repair of tetralogy of Fallot-how much

can we achieve with a single operation? Eur J Cardiothorac Surg. 2015 ;47:535-536.

14. Valente AM, Cook S, Festa P, Ko HH, Krishnamurthy R, Taylor AM, Warnes CA, Kreutzer J, Geva

T. Multimodality imaging guidelines for patients with repaired tetralogy of Fallot: A report from the

American Society of Echocardiography: Developed in collaboration with the society for

cardiovascular magnetic resonance and the society for pediatric radiology. J Am Soc Echocardiogr.

2014;27:111-141

15. Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after

repair of tetralogy of Fallot. Circulation. 2002;106:1374-1378

16. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis MA,

Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM,

Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E, Task Force on the Management of Grown-

up Congenital Heart Disease of the European Society of C, Association for European Paediatric C,

Guidelines ESCCfP. ESC guidelines for the management of grown-up congenital heart disease (new

version 2010). Eur Heart J. 2010;31:2915-2957

17. Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JC, Smith GC, Tat T, Pennell DJ,

Gatzoulis MA. 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;40:2044-2052

18. Li W, Davlouros PA, Kilner PJ, Pennell DJ, Gibson D, Henein MY, Gatzoulis MA. Doppler-

echocardiographic assessment of pulmonary regurgitation in adults with repaired tetralogy of Fallot:

Comparison with cardiovascular magnetic resonance imaging. Am Heart J. 2004;147:165-172

19. Bonello B, Kempny A, Uebing A, Li W, Kilner PJ, Diller GP, Pennell DJ, Shore DF, Ernst S,

Gatzoulis MA, Babu-Narayan SV. Right atrial area and right ventricular outflow tract akinetic length

predict sustained tachyarrhythmia in repaired tetralogy of Fallot. Int J Cardiol. 2013;168:3280-3286


13

20. 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

21. Kilner PJ, Balossino R, Dubini G, Babu-Narayan SV, Taylor AM, Pennati G, Migliavacca F.

Pulmonary regurgitation: The effects of varying pulmonary artery compliance, and of increased

resistance proximal or distal to the compliance. Int J Cardiol. 2009;133:157-166

22. Broberg CS, Aboulhosn J, Mongeon FP, Kay J, Valente AM, Khairy P, Earing MG, Opotowsky AR,

Lui G, Gersony DR, Cook S, Ting JG, Webb G, Gurvitz MZ. Prevalence of left ventricular systolic

dysfunction in adults with repaired tetralogy of Fallot. Am J Cardiol. 2011;107:1215-1220

23. 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;40:1675-1680

24. Valente AM, Gauvreau K, Assenza GE, Babu-Narayan SV, Schreier J, Gatzoulis MA, Groenink M,

Inuzuka R, Kilner PJ, Koyak Z, Landzberg MJ, Mulder B, Powell AJ, Wald R, Geva T.

Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired

tetralogy of fallot enrolled in the indicator cohort. Heart. 2014;100:247-253

25. Diller GP, Kempny A, Liodakis E, Alonso-Gonzalez R, Inuzuka R, Uebing A, Orwat S, Dimopoulos

K, Swan L, Li W, Gatzoulis MA, Baumgartner H. Left ventricular longitudinal function predicts life-

threatening ventricular arrhythmia and death in adults with repaired tetralogy of Fallot. Circulation.

2012;125:2440-2446

26. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA, Khan M, Ho SY, Pennell

DJ, Gatzoulis MA. 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;113:405-413

27. Babu-Narayan SV, Goktekin O, Moon JC, Broberg CS, Pantely GA, Pennell DJ, Gatzoulis MA,

Kilner PJ. 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. 2005;111:2091-2098

28. Giardini A, Lovato L, Donti A, Formigari R, Oppido G, Gargiulo G, Picchio FM, Fattori R. 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. 2006;98:1277-1282

29. Rydman R, Gatzoulis M, Ho S, Ernst S, Swan L, Li W, Wong T, Sheppard M, McCarthy K,

Roughton M, Kilner P, Pennell D, Babu-Narayan S. Systemic right ventricular fibrosis detected by

cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial

arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries. Circ

Cardiovasc Imaging. 2015;8;10.1161/CIRCIMAGING.114.002628

30. 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;55:1721-1728


14

FIGURE LEGENDS

Figure 1

Acute presentation of endocarditis with vegetation (asterisk) related to previous VSD surgical repair patch

leak shown in apical four chamber 2D echocardiography view (A) and magnified from apical five chamber

view (B).

Figure 2

Echocardiography indices TAPSE <15mm, right atrial area ≥ 25cm2, right atrial to left atrial area ratio ≥1.5

and RV systolic over diastolic ratio ≥1.5 are all predictive of survival in Eisenmenger syndrome. Images

show right atrial enlargement (A), prolonged tricuspid regurgitation on Doppler compromising filling

(D=diastole, S=systole in B), biventricular hypertrophy and a flattened interventricular septum (C) and

impaired TAPSE of 11mm (D), in this patient with longstanding Eisenmenger syndrome

Figure 3

Speckle tracking of the LV from apical 4-chamber (A), apical 3-chamber (B) and apical 2-chamber (C)

views in adult patient with repaired anomalous coronary artery from the pulmonary artery (ALCAPA)

syndrome. Despite normal LV ejection fraction, speckle tracking shows LV myocardial dysfunction. The

bullseye plot (D) shows that the anterior and inferior segments are akinetic. Total global longitudinal strain

is -14%.

Figure 4

Large ASD (asterisk) at 3D TOE (A) with relative lack of a posterior rim (B). Fenestrated ASD from left

atrial aspect (C) and from right atrial aspect (D); in these cases 2D echocardiography could potentially

underestimate the size of the overall defect/shunt if the plane sampled is perpendicular to the small defect

only (dotted arrow) .


15

Figure 5

ALCAPA (Ai and Aii) studied with CT preoperatively. Post operative CT (Bi and ii) shows patent left

internal mammary graft to mid left anterior descending artery. (Images courtesy of Dr Mike Rubens). Ao;

aorta, LAD, left anterior descending, LCA; left coronary artery, PA; pulmonary artery, RCA; right coronary

artery.

Figure 6

Anomalous right pulmonary venous drainage to the inferior vena cava creating a curvilinear “scimitar”

silhouette (arrows) on chest x ray (A) with corresponding images from CT (B and C). (Images courtesy of

Dr Mike Rubens).

Figure 7

Contrast enhanced cardiovascular magnetic resonance angiography (CE-CMRA) in an adult presenting with

systemic hypertension due to severe aortic coarctation (A). CT imaging following endovascular stenting (B).

Aneurysm related to endovascular stenting of coarctation studied with cine CMR (C; dotted arrow), and

aneurysm related to Dacron patch coarctation repair studied with CE-CMRA (D; arrow).

Figure 8

CMR (or CT) may be useful in the diagnosis of sinus venosus defects, which can be at the orifice of the

superior (or less commonly inferior caval veins) and to delineate anomalous pulmonary venous drainage.

CMR images showing sinus venosus ASD (asterisk) in A and the anomalous drainage of the right upper

pulmonary vein to superior vena cava (B). IVC; inferior vena cava, LA; left atrium, RA; right atrium,

RUPV; right upper pulmonary vein, PA; pulmonary artery, RCA; right coronary artery, SVC; superior vena

cava.

Figure 9

Diastolic still image from CMR cine pre (A) and post (B) pulmonary valve replacement for pulmonary

regurgitation status post repaired tetralogy of Fallot. Reduction in RV volume and increased LV filling in


16

(B). Late gadolinium enhancement CMR evidence of ventricular fibrosis/scarring is seen in C; block arrows

point to bright areas of scar in the RVOT and dotted arrows to the VSD patch site. Image D is derived from

3D CMR acquisition after segmentation of chambers, outflows and scar using Mimics, Materialise NV

(courtesy of collaboration with Drs Veronica Spadotto and Jennifer Keegan). LV; left ventricle, RV; right

ventricle

Figure 10

Cardiac CT in a patient with RV-PA conduit (A), showing virtually single origin coronary arteries passing

between the aorta and narrow segment of conduit (B-D). (Images courtesy of Dr Mike Rubens). Ao; aorta,

Cx; circumflex, LAD, left anterior descending, LAD; PA; pulmonary artery, RCA; right coronary artery.

Figure 11

CMR images of simple TGA (A,B) and ccTGA (C,D) showing parallel discordant outflows (A,C) and

hypertrophic systemic RV (B,D). Late gadolinium enhancement in TGA with atrial redirection surgery (E)

and in ccTGA (F); bright areas of enhancement within the RV (arrows) suggestive of fibrosis. Ao; aorta,

LA; left atrium, LV; left ventricle, PA; pulmonary artery, PVAC; pulmonary venous atrial compartment,

RV; right ventricle, RA; right atrium.

Figure 12

Images A and B show patent Fontan pathways status post atriopulmonary Fontan. In patient C thrombus has

formed (arrows) due to sluggish flow in the dilated right atrium. In image D, late gadolinium enhancement

CMR evidence of rudimentary endocardial RV fibrosis is seen (arrows). Images E and F show patent total

cavopulmonary pathways (asterisks). Ao; aorta, IVC; inferior vena cava, LA; left atrium, LPA; left

pulmonary artery, LV; left ventricle, RA; right atrium, RPA; right pulmonary artery, SVC; superior vena

cava.


17

Table 1. Cardiac Imaging Goals in selected Adult Congenital Heart Diseases with strengths and weaknesses of different imaging modalities

Modality Echocardiography Cardiovascular Magnetic Resonance Computed Tomography Strengths Widely available

Portable Estimation of pressure gradients Valvular pathology (mechanisms and degrees of dysfunction) Assessing small fine structures (chordal attachments, vegetations) Intracardiac shunts (fenestrations, PFO, VSD)

Quantification of: -ventricular size and function - regurgitant lesions - branch pulmonary artery flow - pulmonary: systemic flow Myocardial viability

Quantification of: -ventricular size and function Aortic pathology Coronary artery imaging

Weaknesses Limited acoustic windows Artifacts from steel implants Relative contraindications in patients with intracardiac leads

Exposure to ionising radiation and iodinated contrast

Lesion Echocardiography Cardiovascular Magnetic Resonance Computed Tomography Left ventricular outflow tract obstruction, including aortic coarctation

Aortic valve morphology, function and pressure gradient Subaortic anatomy (membrane) and pressure gradient Aortic root and ascending aortic anatomy LV size, function and wall thickness

Ascending, transverse and descending thoracic aortic pathology (dilation, multi-level stenosis and aneurysm) Aortic valve morphology LV size, function and mass Aortic collaterals

Ascending, transverse and descending thoracic aortic pathology (dilation, aneurysm) *Particularly useful in the case of prior stents Aortic collaterals

Secundum ASD

Atrial septal anatomy Pulmonary venous anatomy (possible) RV size and function Right ventricular pressure AV valve morphology

Identification of multiple/ fenestrated ASD Identification of rims for device closure Pulmonary:systemic flow ratio Quantification of RV volumes Pulmonary venous anatomy

*rarely used

Sinus venous ASD Atrial septal anatomy Pulmonary venous anatomy (possible) RV size and function Right ventricular pressure

Anomalously draining pulmonary venous connections and drainage Atrial septal anatomy RV size and function Pulmonary:systemic flow ratio

*rarely used


18

Atrioventricular Septal Defect

Ventricular size and function Residual intracardiac shunts AV valve morphology (residual cleft or stenosis), attachments, function Left ventricular outflow tract anatomy/obstruction

Ventricular size and function Residual intracardiac shunts Pulmonary:systemic flow ratio AV valve morphology (residual clefts), attachments, function Valvular regurgitant fractions Left ventricular outflow tract anatomy and obstruction

*rarely used

Tetralogy of Fallot repair

RV size and function RVOT, branch PAs Residual intracardiac shunts Right ventricular pressure Tricuspid regurgitation Pulmonary regurgitation LV size and function Aortic root size

RV size and function Regional wall motion RVOT aneurysms Branch PA anatomy and flow Pulmonary: systemic flow ratio Pulmonary regurgitant fraction LV size and function Aortic root and ascending aortic size Aortic-to-pulmonary artery collaterals Origin and proximal course of coronary arteries

*In selected cases where CMR is contraindicated Ventricular size and function Branch PA anatomy Aortic root and ascending aortic size Aortic-to-pulmonary artery collaterals Origin and proximal course of coronary arteries RV to PA conduit calcification and proximity to coronary arteries

Transposition of the great arteries, atrial switch procedure

Systemic RV size and function Systemic and pulmonary venous pathways (baffle leaks or obstructions) Residual intracardiac shunts Tricuspid regurgitation LV size & function LVOT obstruction (pulmonary stenosis)

Systemic RV size and function Systemic and pulmonary venous pathways (baffle leaks or obstructions) Residual intracardiac shunts Tricuspid regurgitant fraction LV size and function LVOT obstruction (pulmonary stenosis) Origins and proximal courses of the coronary arteries

*In selected cases where CMR is contraindicated Ventricular size and function Systemic and pulmonary venous pathways (baffle leaks or obstructions) Residual intracardiac shunts LVOT obstruction (pulmonary stenosis) Origins and proximal courses of the coronary arteries

Transposition of the great arteries, arterial switch procedure

LV size and function Residual intracardiac shunts Main PA and proximal branch PA stenosis Neo-aortic root dilation and regurgitation

LV size and function Residual intracardiac shunts Main PA and proximal branch PA stenosis Neo-aortic root dilation & regurgitation Origin and proximal course of coronary

*In selected cases LV size and function Residual intracardiac shunts Main PA and proximal branch PA stenosis Neo-aortic root dilation and regurgitation


19

arteries Myocardial stress perfusion

Origin and proximal course of coronary arteries

Congenitally corrected transposition of the great arteries

Systemic RV size and function Residual intracardiac shunts Tricuspid valve morphology and regurgitation Aortic regurgitation LV (subpulmonary) size and function LVOT obstruction (pulmonary stenosis)

Systemic RV size and function Residual intracardiac shunts Tricuspid valve morphology & regurgitation Aortic regurgitation LV (subpulmonary) size and function LVOT obstruction (pulmonary stenosis)

*In selected cases Ventricular size and function Residual intracardiac shunts Tricuspid valve morphology & regurgitation Aortic regurgitation LVOT obstruction (pulmonary stenosis)

Single Ventricle, Fontan Procedure

Ventricular size and function Atrioventricular valve regurgitation Fontan pathway patency Branch PA caliber LVOT obstruction Aortic regurgitation

Ventricular size and function Atrioventricular valve regurgitant fraction Fontan pathway patency Branch PA caliber and flow LVOT obstruction Aortic regurgitation fraction Pulmonary venous compression Aortic-to-pulmonary collaterals Systemic-to-pulmonary venous collaterals

*In select cases Ventricular size and function Atrioventricular valve regurgitant fraction Fontan pathway patency Branch PA calibre LVOT obstruction Pulmonary venous compression

Abbreviations: ASD; atrial septal defect, AV; atrioventricular, LV; left ventricle. LVOT; left ventricular outflow tract obstruction, PA; pulmonary artery, PFO;

patent foramen ovale, PS; pulmonary stenosis, RV; right ventricle, RVOT; right ventricular outflow tract, VSD; ventricular septal defect


20

FIGURES

Figure 1

A B

*


21

Figure 2

A

D C

D

S

B


22

Figure 3

A B

C D


23

Figure 4

A B

C D

*

*


24

Figure 5

A i A ii

B i B ii

Ao PA

Ao PA graft

to

LAD

graft

to

LAD

LCA RCA

RCA LCA

PA Ao


25

Figure 6

A B C


26

Figure 7

A B

C D


27

Figure 8

RA

LA

*

SVC

IVC

A

RUPV SVC

B


28

Figure 9

A

C

RV

LV

RV

LV

RV

LV

B

RV

LV

D


29

Figure 10

RCA

RCA

Cx

LAD

LAD

Cx

Ao P

A

PA

PA

A B

C D

RCA

LAD

Cx

Ao Ao

Ao

RV PA


30

Figure 11

Ao

PA

RV

LV

RA LA

RV LV

C D

A

Ao PA

RV LV

B

PVAC

LA

RV

LV

Ao

PA

RV LV RA

LA

RV LV

E F


31

Figure 12

LPA

RPA

RA LV

RA

SVC

IVC

RA

LA

LV

*

*

IVC

Ao

LPA

A B

C D

E F

RA RA LV

LA

LV

imaging of congenital heart disease in adultsimaging of congenital heart disease in adults...

Documents