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rchives of Cardiovascular Disease (2016) 109, 120—127

Available online at

ScienceDirectwww.sciencedirect.com

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uantification of systemic right ventricle bychocardiographyuantification du ventricule droit systémique par échocardiographie

Xavier Iriarta,b,∗, Francois Roubertieb,c,Zakaria Jalal a,b, Jean-Benoit Thamboa,b

a Congenital heart disease unit, Bordeaux university hospital, Pessac, Franceb Institut de rythmologie et de modélisation LIRYC, université de Bordeaux, Inserm 1045,Bordeaux, Francec Surgical centre for congenital heart disease, Bordeaux university hospital, Pessac, France

Received 4 September 2015; received in revised form 5 November 2015; accepted 11 November2015Available online 2 February 2016

KEYWORDSSystemic rightventricle;Echocardiography;Systlic function

Summary Improvements in cardiac imaging have recently focused a great interest on theright ventricle (RV). In patients with congenital heart disease, the right ventricle (RV) maysupport the systemic circulation (systemic RV). There are 2 different anatomic conditions pro-viding such physiology: the congenitally corrected transposition of the great arteries (ccTGA)and the TGA surgically corrected by atrial switch. During the last decades, evidence is accu-mulating that progressive systemic RV failure develops leading to considerable morbidity andmortality. Various imaging modalities have been used to evaluate the systemic RV, but echocar-diography is still predominantly used in clinical practice, allowing an anatomic and functionalapproach of the systemic RV function and the potential associated anomalies. The goal of thisreview is to offer a clinical perspective of the non-invasive evaluation of the systemic RV byechocardiography.

© 2015 Elsevier Masson SAS. All rights reserved.

Abbreviations: 2D, two-dimensional; 2DE-KBR, 2Dechocardiography with 3D knowledge-based reconstruction; 3D, three-dimensional;cTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease; FAC, fractional area change; IVA, isovolumicyocardial acceleration; LV, left ventricle/ventricular; MRI, magnetic resonance imaging; RT3DE, real-time 3D echocardiography; RV, right

entricle/ventricular; RVEF, right ventricular ejection fraction; SR, strain rate; STE, speckle-tracking echocardiography; TAPSE, tricuspidnnular plane systolic excursion; TDI, tissue Doppler imaging; TGA, transposition of the great arteries; TR, tricuspid regurgitation.∗ Corresponding author at: Congenital Heart Disease Unit, Bordeaux University Hospital, Haut-Lévêque, Magellan avenue, 33600 Pessac,rance.

E-mail address: xavier.iriart@chu-bordeaux.fr (X. Iriart).

http://dx.doi.org/10.1016/j.acvd.2015.11.008875-2136/© 2015 Elsevier Masson SAS. All rights reserved.

Quantification of systemic right ventricle by echocardiography 121

MOTS CLÉSVentricule droitsystémique ;Échographie ;Fonction systolique

Résumé Les améliorations de l’imagerie cardiaque ont permis de se focaliser récemment surl’étude du ventricule droit. Chez certains patients porteurs d’une cardiopathie congénitale, leventricule droit peut se retrouver en position systémique. C’est le cas des patients porteursd’une double discordance et patients porteurs d’une transposition des gros vaisseaux opérés parun switch à l’étage atrial. Durant les dernières décennies, les données scientifiques ont montréune dégradation progressive du ventricule droit systémique, qui est associée à une morbiditéet une mortalité importante. Différentes modalités d’imagerie ont été utilisées pour évaluerle ventricule droit systémique mais l’échographie reste la méthode de choix dans l’activitéquotidienne, permettant une analyse anatomique et fonctionnelle. L’objectif de cette revueest d’offrir une perspective clinique de l’évaluation non invasive du ventricule droit systémiquepar échocardiographie.© 2015 Elsevier Masson SAS. Tous droits réservés.

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Introduction

Improvements in cardiac imaging have recently focusedgreat attention on the right ventricle (RV), emphasising itsimportance in cardiac physiology. The RV has been studiedin many physiopathological conditions, such as pulmonaryhypertension and heart failure related to left ventricular(LV) dysfunction.

Specific congenital heart diseases (CHD) may provide anoriginal condition where the RV is pumping through the sys-temic circulation. In these anatomical and haemodynamicconditions, the RV is called systemic RV (systemic meaningpumping through the systemic circulation as opposed to thepulmonary circulation). There are two different anatomicalconditions that provide such physiology.

The first condition is congenitally corrected transposi-tion of the great arteries (ccTGA), where the left atrium isconnected to the RV, pumping in the aorta, and where theright atrium is connected to the left ventricle (LV), pump-ing in the pulmonary artery branches. The other conditionis related to atrial switch, which is the surgical treatmentdeveloped in the late 1950s by Ake Senning [1] and in themid-1960s by William Mustard [2] for surgical correction oftransposition of the great arteries (TGA). In terms of livebirth incidence, TGA is the more common condition (TGA1:3100 live births vs ccTGA 1:33,000), but the absolute num-ber of Mustard and Senning patients is decreasing becauseatrial redirection procedures for TGA were superseded bythe arterial switch operation in the 1980s. As a consequence,almost all patients with TGA and atrial redirection reachadulthood.

For both populations, right ventricular (RV) failure isan important long-term concern, leading to severe latecomplications [3—5]. Various imaging modalities have beenused to evaluate the systemic RV, including angiography,radionuclide imaging and magnetic resonance imaging(MRI) [6]. Nevertheless, in clinical practice, echocardiog-raphy is still used predominantly for the assessment of RVfunction, as it is non-invasive, widely available, relativelyinexpensive and has no adverse side effects. However,because of complex geometry, the assessment of systemicRV function by echocardiography has remained mostly

qualitative. Advances in digital echocardiography allow fora more refined assessment of the RV, as demonstrated inpatients with pulmonary arterial hypertension [7] and other

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linical conditions. These novel echocardiography variablesay also be valuable in the functional assessment of the

ystemic RV [8]. The goal of this review is to offer a clinicalerspective of the non-invasive evaluation of the systemicV by echocardiography.

chocardiographic assessment of systolicunction in the systemic RV

hy the RV is different

y analogy with the LV, RV ejection fraction (RVEF) is consid-red to be the marker of RV function. However, attemptingo extend this to the RV has been problematic. The shapef the RV does not allow the use of geometric formulae toalculate the RVEF. A range of echocardiographic variablesas therefore been developed to evaluate RV function in theubpulmonary position, especially simple measurements ofong-axis excursion, giving rapid and unambiguous results8—10], which has proven utility as a measure of LV func-ion in patients with coronary artery disease, valve diseasend heart failure [11,12]. The technique is equally straight-orward for the RV, and is especially valid because most ofhe RV myocardial fibres are arranged longitudinally. Theseariables have been compared with other methods of calcu-ating the RVEF, especially cardiac MRI, which is consideredo be the gold standard for RV functional assessment [13].ogically, these echocardiographic variables have been usedubsequently for systemic RV functional assessment (Fig. 1).

asic longitudinal function variables

he first studies focused on the analysis of longitudinalyocardial fibres using mainly tricuspid annular plane sys-

olic excursion (TAPSE), based on the anatomical hypothesishat the majority of RV myocardial fibres originate at thepex of the heart and insert into the right atrioventricularunction, such that the main bulk of the RV myocardiums composed of longitudinally arranged fibres [14]. Der-ick et al. showed that systemic RV long-axis function

ormal subpulmonary RV or the systemic LV. The authorsypothesized that impaired systemic RV longitudinal func-ion reflected the response of the longitudinally arranged

122 X. Iriart et al.

Figure 1. Longitudinal variables commonly used for right ventricular systolic function assessment (panels A—D). A. Tricuspid annular planes iew ai view

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yocardial fibres to chronic increased afterload [8], buthey underlined that the systemic RVEF could remainemarkably constant over long-term follow-up [16], and thathere were no ‘‘normal’’ RVEF values under these circum-tances. TAPSE was further compared with cardiac MRI whenhe technique became the gold standard for RVEF measure-ent in the early 2000s. Lissin et al. [15] showed rather good

orrelation between TAPSE and MRI-based systemic RVEF in amall cohort of patients with TGA and previous atrial switchrocedure.

asic longitudinal function variables

issue Doppler imaging and tissueoppler-derived strain and strain-rate imagingissue Doppler imaging (TDI) is an echocardiographicechnique that uses Doppler principles to measure theelocity of myocardial motion. TDI can be performed inulsed-wave and colour modes. Pulsed-wave TDI is usedo measure peak myocardial velocities, and is particularlyell suited to the measurement of long-axis ventricularotion, because the longitudinally oriented endocardialbres are most parallel to the ultrasound beam in thepical views, and it is a good surrogate measure of overallongitudinal RV contraction and relaxation. Strain and strainate (SR) are TDI-derived modalities. SR measures the rate

f deformation of a tissue segment, and is measured in s−1.eak systolic SR represents the maximal rate of deformationn systole. Strain is obtained by integrating the magnitudef deformation between end-diastole used as a reference

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nd M-mode. B. S’ tissue Doppler imaging (TDI) of the tricuspid ring. D. Two-dimensional strain imaging in the A4C view.

oint and end-systole. Isovolumic myocardial accelerationIVA) is also a TDI-based index of contractile function, whichas been applied to RV function assessment because itas shown experimentally not only to measure contractile

unction accurately, but also to be relatively independentf acute changes in ventricular preload and afterload [16].ogel et al. confirmed reduced systolic contractile functionn the systemic RV of Mustard and Senning patients. Thejection phase indexes, S wave velocity and acceleration,ere both reduced [17] compared with both systemic LVnd subpulmonary RV in controls. Furthermore, in a subsetf 12 patients with simultaneous conductance cathetereasurements, IVA correlated with the change in elastance

ssociated with dobutamine stress [17]. These data wereonfirmed in a population of asymptomatic patients withcTGA compared with subpulmonary RV in normal patientsssessed by peak systolic SR and peak systolic strain [18].

Strain and SR imaging are useful for differentiating activend passive movement of myocardial segments, and to quan-ify and evaluate components of myocardial function, suchs longitudinal myocardial shortening, which are not visu-lly assessable; they allow comprehensive assessment ofyocardial function, which is of particular importance for

ystemic RV assessment, considering the specific haemody-amic conditions. However, TDI-derived strain and SR haveeveral disadvantages related to angle-dependency and theimited spatial resolution imposed by imaging at high tempo-al resolution. Other disadvantages of the TDI-derived strain

nd SR imaging techniques are the time-consuming stepsor data acquisition and processing and the need for experteaders. Taking into account all of the factors mentioned

Quantification of systemic right ventricle by echocardiography 123

Figure 2. Global variables for right ventricular (RV) systolic function assessment. A. Fractional area change, calculated by dividing thedifference between the RV end-diastolic surface and the RV end-systolic surface in the apical four-chamber view by the RV end-diastolicsurface. B. Right ventricular real time three-dimensional (3D) reconstruction using Tomtec software: top, mesh view; bottom, exampleof the stepwise process of RV reconstruction by 3D echocardiography, where the cavity area is optimized in the three orthogonal viewsand landmarks are identified. C. RV knowledge-based reconstruction model with different views enables evaluation of the 3D shape of thereconstruction.

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above, it is not surprising that TDI-derived strain and SRmeasurements are not highly reproducible [19], as with IVA,which carries even higher variability [20]. As a consequence,even if these variables are very interesting in terms of help-ing us to understand cardiac physiology, their applicabilityin clinical practice remains to be proved.

Speckle-tracking-derived two-dimensionalstrain imagingSpeckle-tracking echocardiography (STE) is a newer tech-nique for obtaining strain and SR measurements, whichanalyses motion by tracking speckles (natural acoustic mark-ers) in the two-dimensional (2D) ultrasonic image. Thesemarkers (‘‘stable’’ speckles) within the ultrasonic image aretracked from frame to frame. Special software allows spatialand temporal image processing, with recognition and selec-tion of such elements on ultrasound images. The geometricshift of each speckle represents local tissue movement.By tracking these speckles, strain and SR can be calcu-

lated. The advantage of this method is that it tracks intwo dimensions, along the direction of the wall, not alongthe ultrasound beam, and thus is angle independent. STErequires only one cardiac cycle to be acquired, but strain

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nd SR data can be obtained only with high-resolution imageuality at a high frame rate. The need for high image qual-ty is a potential limitation to routine clinical applicabilityn all patients. STE was recently introduced for assessmentf RV function in patients with TGA [21,22], in the con-ext of TDI-derived strain and SR imaging, suggesting thateformation-based variables may provide incremental prog-ostic information over standard variables in this high-riskopulation. In concordance with earlier studies that used TDI17,18], it has been reported that STE-derived deformationariables of the RV are depressed in patients with TGA andtrial switch (Fig. 2). A recent study confirmed these prelim-nary results, showing that systemic RV global longitudinaltrain was able to discriminate TGA patients after atrialwitch with and without a cardiac MRI systemic RVEF ≥ 45%23].

The conclusions of these studies using advanced imagingechnology agree with the conclusions of the first studiessing basic variables. Evidently, though, there are no clear‘normal’’ RVEF values under these circumstances, and lon-

itudinal function variable values are probably different tohose applied to subpulmonary RV. Furthermore, it remainsncertain whether altered markers of longitudinal functionimply reflect the chronic response of the RV to its loading

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onditions. It has been shown that RVEF, for example, canemain remarkably constant over long-term follow-up [24],nd prediction of RV failure is difficult.

The question of the prognostic value of deformationariables of the systemic RV in adults with TGA has beenecently addressed by Kalogeropoulos et al. [25]. Theseuthors observed that RV global longitudinal strain (with aut-off of −10%) had the strongest association with clini-al events, followed by RV global longitudinal SR and globalarly diastolic SR. A similar finding was reported by Dillert al. [26], who suggested that biventricular 2D longitudi-al strain was reduced in adult patients with systemic RV,nd was related to adverse clinical outcome in this settingwith a cut-off value of −13.3%, providing 72% sensitiv-ty and 63% specificity in identifying patients with adverselinical outcome). Systemic and subpulmonary myocardialunctions appear to be inter-related in patients with TGA,uggesting adverse ventriculoventricular interaction, andhe relationship between systemic and subpulmonary ven-ricular functions was found to be most pronounced inatients with ccTGA and pulmonary stenosis.

A clear difference has to be made between severe longi-udinal function impairment and mild impairment. Indeed,elated to chronic pressure overload, severe RV dilatationnd hypertrophy are observed in all patients, as an adap-ive process. Pettersen et al. [27] have shown that theystemic RV contraction pattern could be shifted towardhe LV contraction pattern, with predominant circumfer-ntial shortening and a relative decrease in longitudinalhortening. Based on this theory, a mild decrease in longi-udinal function would be more an adaptive process than aeal impairment of function. Recently, a cut-off to deter-ine the lower limit of normal longitudinal function of

he systemic RV has been proposed [25,26], and it has toe underlined that it differs from that usually used forubpulmonary RV. However, there is no doubt that severempairment of longitudinal 2D strain is a marker of global RVunction impairment, and could be used a prognostic factorn serial quantitative assessment [25,26]. This pathophys-ological adaptation has also been recently demonstratedarly in life in a patient with hypoplastic left heart syndromeost-Norwood stage 1 operation.

Recently, others and we [28,29] tried to redefine the rolef echocardiography in the functional assessment of theystemic RV in clinical practice. We confirmed an overalleak correlation of conventional echocardiographic varia-les of systolic RV function in adult patients with systemicV compared with cardiac MRI-derived RVEF. For some ofhe well-established conventional surrogates of RV func-ion, such as TAPSE and myocardial performance index, noignificant correlation was found with cardiac MRI resultsn this setting [28,29]. In contrast to the study of Lissint al. [15], who found a moderate correlation (r = 0.63) in

small number of patients (n = 18), we did not find accep-able correlation between global and longitudinal functionalariables compared with MRI. Our cohort was larger thanhat in the study by Lissin et al., and our population wasore heterogeneous in terms of anatomical distribution

etween complex and simple TGA. Furthermore, as demon-trated by MRI measurements, RV end-diastolic volume wasarger in our population, resulting in a larger RV diameter,hich can complicate echocardiographic assessment of the

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V. Our findings were further confirmed by Khattab et al.29]. The main hypothesis to explain the lack of correla-ion between TAPSE and MRI-derived RVEF is that TAPSEnd lateral tricuspid annular motion velocity are accurateor assessing systolic function in subpulmonary RVs, withyocytes predominantly oriented and contracting in the

ongitudinal direction. Under these circumstances, the lat-ral tricuspid annular motion velocity and tricuspid valvennular systolic excursion reflect an important aspect ofhe RV contraction pattern. In a subaortic RV, however,ircumferential wall shortening might be more predomi-ant than longitudinal shortening, leading to a systemic RVontraction pattern that resembles the contraction patternf an anatomical LV [27]. Thus, the measures of longitudi-al shortening or shortening velocity might be less likely toepresent global RV function in a subaortic compared with aubpulmonary RV, especially for standard variables (TAPSE,ricuspid annulus tissue velocities) that reflect mainly theong-axis function of the laterobasal RV free wall. GlobalTE-longitudinal has major advantages over the above men-ioned variables because it includes medial and apicalegments of the systemic RV, and can differentiate betweenctive and passive movement of myocardial segments, toetter quantify myocardial longitudinal shortening. How-ver, it has to be kept in mind that RV deformation isurrently only assessed in the longitudinal direction withchocardiography, and leaves deformation in other dimen-ions undefined. There are very scarce data on deformationmaging of the systemic RV in the other directions bychocardiography [30], but they tend to confirm a shift from

longitudinal to a circumferential contraction pattern. Theain reasons for the limited availability of this information

s the difficulty in meaningfully encompassing the retroster-al and dilated RV in the short-axis view in TGA patients,nd the rather poor reproducibility previously reported forhe LV.

onventional global variables

he systemic RV is characterized by important remodelling,ith spherical reconfiguration close to the LV shape, if wexclude the RV outflow tract. One of the first echocar-iographic descriptions of systemic RV systolic functioname from adult patients with Mustard baffle repair, usingethods of volume and RVEF estimation derived from LVeasurements (single-plane methods of ventricular vol-

me and area estimation for RVEF calculation) comparedith radionucleotide angiography [31]. The correlation withucleotide angiography regarding RVEF was relatively weak,nd the main problem identified by the authors was theifficulty in obtaining complete border detection for thentirety of complex geometric shape of the RV. One mustote that the system used at that time (Hewlett-Packardonos 2000 echocardiography system; Agilent Technologies,alo Alto, CA, USA) was rudimentary compared with currentystems. The major improvements in ultrasound techniquesogether with a better understanding of systemic RV remod-lling and mechanics have recently emphasized the value of

lobal function variables. The use of fractional area changeFAC) [23,25,28] and even LV-derived global variables, suchs Simpson’s method, to assess global systolic function semi-uantitatively, have been applied to systolic RV function

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Table 1 Cut-offs for systolic right ventricular functionvariables, based on receiver operating characteristiccurve for prediction of cardiac magnetic resonanceimaging-derived systemic right ventricular ejection frac-tion < 45% [23,29] and Cox models for prediction ofclinical events [25,26].

Variable Cut-off

TAPSE (mm) < 14 [29]Pulsed Doppler velocity

at the annulus (cm/s)< 10 [29]

IVA (m/s2) 1.0 [17]Systemic RV GLS (%) > −10 to −14.5 [21—23]FAC (%) < 29.5 to 33 [23,29]3D RVEF (%) < 45 [33]

3D RVEF: three-dimensional right ventricular ejection fraction;FAC: fractional area change; IVA: isovolumic acceleration;RV GLS: right ventricular global longitudinal strain; TAPSE:tricuspid annular plane systolic excursion.

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assessment [22,28]. FAC is measured in the four-chamberview (Fig. 2A); thus, the contribution of the RV outflow tractto global RV function is ignored. However, the circumfer-ential and radial contraction pattern of the RV free wallis incorporated into the analysis of ventricular function.Khattab et al. [29] found that FAC had the best sensitiv-ity (77%) for distinguishing between normal and reduced RVfunction among conventional variables, and was the bestvariable after speckle-tracking systolic and diastolic varia-bles for predicting clinical outcome. These results wererecently confirmed by Lipczynska et al. [23]. FAC has alsodemonstrated its value in repaired tetralogy of Fallot witha severely dilated subpulmonary RV [32].

Advanced global variables

Real time three-dimensional echocardiographyTo overcome the problem of geometric assumptions andapical foreshortening, real-time three-dimensional echocar-diography (RT3DE) was developed, allowing the fastacquisition of a pyramidal data set that contains the entireRV (TomTec Imaging Systems GmbH, Munich, Germany). Inexperimental settings, this imaging modality was success-fully applied to calculate RV volume and RVEF in bothhealthy volunteers and paediatric patients with CHD. Adedicated study has yet to investigate the feasibility andreproducibility of this technique for systemic RV volumeand function assessment. We have recently shown that inadult patients with repaired tetralogy of Fallot, there wasgood correlation between RT3DE and MRI for RVEF, but alarge underestimation of RV volumes, especially in severelydilated subpulmonary RV, with great difficulties in encom-passing the entire RV in the pyramidal sector size [32]. Wecan presume that the same limitation would be faced forsystemic RV assessment with this technique (Fig. 2B).

2D echocardiography with 3Dknowledge-based reconstruction2D echocardiography with 3D knowledge-based reconstruc-tion (2DE-KBR) has also been evaluated in the measurementof systemic RV volumes and function (VentriPoint Diagnos-tics Ltd., Seattle, WA, USA). This imaging tool is basedon a piecewise smooth subdivision surface reconstructiontechnology that creates a ‘‘knowledge base’’ for the RVusing image data acquired from combinations of views, andhas been validated against MRI. Kutty et al. [33] recentlyreported on the feasibility of 2DE-KBR for the evaluationof the systemic RV, and showed the clinical feasibility ofquantifying systemic RV volumes and function in adolescentsand young adults with repaired TGA, and good agreement ofmeasurements with MRI (Fig. 2C). The different echocardio-graphic modalities are summarized in Table 1.

Associated features for assessment ofsystemic RV function

Tricuspid regurgitation

There are extensive data supporting the concept that tri-cuspid regurgitation (TR) is the key driver of progressive

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ystemic RV dysfunction. Several studies have demonstratedn association between the severity of TR and RV systolicysfunction [3,5]. By comparison, patients with isolatedcTGA with minimal TR might remain asymptomatic withreserved ventricular function for decades [34]. Anatomicalalformations of the tricuspid valve are the most com-on intracardiac abnormality associated with ccTGA. Theost frequent problem is an Ebstein-like thickening of the

hords that anchor the leaflets to the ventricle [35]. In otheratients with a subaortic RV and more anatomically normalricuspid valves, the tendency to develop TR might relateo anatomical differences between the mitral and tricuspidalves and their subvalvar apparatus, affecting their relativebilities to withstand systemic ventricular pressures and toaintain competence as the systemic ventricle dilates. It

s also clear that tricuspid valve function in patients withcTGA appears to be adversely affected by surgical repair ofssociated lesions, especially ventricular septal defect clo-ure, which can create anatomical distortion [34]. In ccTGA,he usual vicious circle occurs whereby the inability of the RVo cope with significant TR leads to decreased contractilitynd annular dilation that in turn exacerbate the degree ofR, but this occurs at an accelerated rate compared with annatomical LV with similar degrees of mitral incompetence.s a consequence, careful monitoring of TR has to be per-ormed in the longitudinal follow-up by echocardiography.urthermore, in the setting of systolic function assessment,he change in loading conditions related to chronic TR is anmportant feature to take into account.

ystemic RV dyssynchrony

ntraventricular dyssynchrony in the setting of systemicV has been studied using electromechanical and elec-rosystolic intraventricular delay measurement by TDI andTE-derived strain imaging [36]. The presence of dyssyn-

hrony between the ventricles and within the systemicV in patients with TGA after atrial redirection was pre-iously reported by Chow et al. [21]. We found that

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entricular dyssynchrony was more dominantly present inatients receiving ventricular pacing in the non-systemici.e. left) ventricle [36]. As asynchronous electromechan-cal activation may be one cause of RV dysfunction [37],ts identification should be sought during echocardiographicssessment of systemic RV function. Favourable effects ofardiac resynchronization therapy have been reported in amall series of patients with TGA [38], and echocardiographyas a certain value in the management of the resynchroniza-ion, especially in patients with a pacemaker.

ystemic RV ischaemic pattern

V wall motion abnormalities have been described andinked with a reduction in systolic function [39]. The causef wall motion abnormalities is not clear, but their presenceas been described preoperatively in TGA patients [40],resumably resulting from preoperative myocardial dam-ge. Additionally, it has been demonstrated using nuclearedicine data that resting and induced RV perfusion defects

re related to global function [41,42]. Lubiszewska et al.41] demonstrated that stress-induced perfusion abnormal-ties correlated with increased age, later repair and lowerVEF and LVEF. Li et al. [43] performed stress echocardiogra-hy in TGA patients with atrial redirection, and showed thatystemic RV function was impaired during pharmacologicaltress and was related to limited exercise capacity.

valuation of systemic RV diastolicunction

he prognosis and exercise capacity of patients with sys-emic RV and atrial redirection are not only affected byystolic dysfunction but also by diastolic function. Riecht al. [24] showed, using radionuclide ventriculography andchocardiography, that the abnormal filling of the ventri-les was not only related to impaired diastolic properties ofhe ventricles, but was mainly because of filling abnormal-ties through surgical baffles; these decrease the capacityf the systemic venous atrium and diminish the ability oftrial contraction to boost ventricular filling. This abnormalentricular filling seems to remain constant over time [24].he findings were in agreement with the reported diminu-ion of early LV filling in patients after atrial redirection,ssessed by jugular venous flow measurement using Dopplerltrasound [44]. This hypothesis was further confirmed byoad-independent indexes of ventricular function measuredy a conductance catheter during dobutamine infusion,hich showed that abnormal stroke volume responses dur-

ng dobutamine stress were not explained by reducedhronotropic or contractile responses, but were associatedith fixed ventricular filling rates [45].

onclusion

lthough the functional status of many adults with sys-

emic RV is good, reduced exercise capacity is the norm.his finding may relate to many different factors, suchs impaired systemic ventricular function, TR, impairedapacitance and conduit function of the intra-atrial baffles

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r chronotropic incompetence. Echocardiography remainshe daily-life imaging modality for systemic RV functionssessment. Because of geometric changes, presumed con-ractility pattern shift and retrosternal position in TGA withtrial redirection, conventional echocardiographic varia-les are limited for RV function assessment compared withRI. Nevertheless, echocardiography remains the techniquef reference for dyssynchrony and TR assessment. Newchocardiographic deformation imaging modalities haveeen proved to be relevant for left heart disease prognosis,nd seem promising for the assessment of systemic RV func-ion. To make a valuable estimation of RV systolic functionsing echocardiography, a combination of multiple variableshould be performed, in association with diastolic functionnd TR assessment.

isclosure of interest

he authors declare that they have no competing interest.

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Quantification of systemic right ventricle by echocardiograp

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