Cardiovascular magnetic resonance findings in a pediatric population with isolated left ventricular non-compaction.

2.1 MB·English

by  Uribe, Sergio

Uribeetal.JournalofCardiovascularMagneticResonance2012,14:9
http://www.jcmr-online.com/content/14/1/9
RESEARCH Open Access
Cardiovascular magnetic resonance findings in a
pediatric population with isolated left ventricular
non-compaction
Sergio Uribe1,3*, Lina Cadavid1, Tarique Hussain5, Rodrigo Parra1, Gonzalo Urcelay4, Felipe Heusser4,
Marcelo Andía5, Cristian Tejos2,3 and Pablo Irarrazaval2,3
Abstract
Background: Isolated Left Ventricular Non-compaction (LVNC) is an uncommon disorder characterized by the
presence of increased trabeculations and deep intertrabecular recesses. In adults, it has been found that Ejection
Fraction (EF) decreases significantly as non-compaction severity increases. In children however, there are a few data
describing the relation between anatomical characteristics of LVNC and ventricular function. We aimed to find
correlations between morphological features and ventricular performance in children and young adolescents with
LVNC using Cardiovascular Magnetic Resonance (CMR).
Methods: 15 children with LVNC (10 males, mean age 9.7 y.o., range 0.6 - 17 y.o.), underwent a CMR scan.
Different morphological measures such as the Compacted Myocardial Mass (CMM), Non-Compaction (NC) to the
Compaction (C) distance ratio, Compacted Myocardial Area (CMA) and Non-Compacted Myocardial Area (NCMA),
distribution of NC, and the assessment of ventricular wall motion abnormalities were performed to investigate
correlations with ventricular performance. EF was considered normal over 53%.
Results: The distribution of non-compaction in children was similar to published adult data with a predilection for
apical, mid-inferior and mid-lateral segments. Five patients had systolic dysfunction with decreased EF. The number
of affected segments was the strongest predictor of systolic dysfunction, all five patients had greater than 9
affected segments. Basal segments were less commonly affected but they were affected only in these five severe
cases.
Conclusion: The segmental pattern of involvement of non-compaction in children is similar to that seen in adults.
Systolic dysfunction in children is closely related to the number of affected segments.
Keywords: isolated left ventricular non-compaction, cardiomyopathy, spongy myocardium, ventricular performance,
prominent trabeculations
Background LVNC may be present as an isolated finding (isolated
LeftVentricularNon-Compaction(LVNC)ismorpholo- LVNC) or in association with other congenital heart or
gically characterized by numerous prominent trabecula- neuromusculardisorders[2,3].Distributionofleftventri-
tions and deep intertrabecular recesses [1]. It is also cularsegmentswithnon-compaction(NC)canvaryfrom
knownas“spongymyocardium”or“persistentembryonic one patient to another, but it often involves the apical,
myocardium”,anditisprobablysecondarytoanarrestin mid-inferior, and mid-lateral myocardial segments [4].
the normal process of myocardial compaction during The clinical manifestations, including age of onset, are
fetallife. variable. Functional cardiac status may range from
absence of symptoms to severe cardiac disability, some-
times leading to heart transplantation or death [5,6].
*Correspondence:[email protected]
1RadiologyDepartment,PontificiaUniversidadCatólicadeChile.Marcoleta Clinicalfindingsincludeevidenceofsystolicanddiastolic
367,HospitalClinicoUniversidadCatolica,Santiago8330024,SantiagoChile dysfunction,systemic embolism,andsevere arrhythmias
Fulllistofauthorinformationisavailableattheendofthearticle
©2012Uribeetal;licenseeBioMedCentralLtd.ThisisanOpenAccessarticledistributedunderthetermsoftheCreativeCommons
AttributionLicense(http://creativecommons.org/licenses/by/2.0),whichpermitsunrestricteduse,distribution,andreproductionin
anymedium,providedtheoriginalworkisproperlycited.
Uribeetal.JournalofCardiovascularMagneticResonance2012,14:9 Page2of9
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[7]. Because of the prominent trabeculations, subendo- Methods
cardialischemiamayalsoresultfromisometriccontrac- Study Population
tion of the endocardium and myocardium within the Institutional Review Board Approval was obtained. All
deepintertrabecularrecesses;thismayleadtomyocardial patients or guardians gave permission to use the images
scaring,leftventriculardysfunctionandarrhythmias[8]. for any research purpose when the MR scan was carried
Echocardiography is the most commonly used imaging out.Aretrospective analysiswasperformedontheCMR
modality for the diagnosis of LVNC. In some cases how- imagesofapediatricpopulationof15childrenandyoung
ever, poor acoustic windows could mislead the diagnosis adolescents (10 males, meanage9.7 yearold,range0.6 -
of this disease, resulting in an erroneous label such as 17yearold,SD=5.2).Theywerereferredto ourRadiol-
dilated or hypertrophic cardiomyopathy [8]. Cardiovas- ogyDepartment,between2005and2008,forperformance
cular Magnetic Resonance (CMR) may outperform of a CMR scan for confirmation of LVNC. All of them
echocardiography in defining the morphology and hadbeenpreviouslyevaluatedwithechocardiographyand
extension of myocardial non-compaction [2]. CMR can allpatientsmetbothechocardiographic andMRIcriteria
also provide excellent delineation of the abnormal trabe- for LVNC. Symptoms such as shortness of breath, dys-
culations owing to its higher contrast and resolution. pnea, palpitations, chest pain, exercise intolerance, and
This allows a thorough identification as well as quantifi- presenceofarrhythmiaswerealsorecorded.
cation of the extent of non-compaction. Furthermore, Insixcases, MRimagingwasperformedundergeneral
CMR also has the ability of detecting myocardial viabi- anesthesia. This procedure was done using a DRÄGER
lity [9,10], which has been previously described in Titusanaesthesiadevicewithpressure-controlledventila-
patients with LVNC [11]. tion(routineprotocolforyoungoruncooperativechildren
Different echocardiographic and CMR measurements atourinstitution).Aftergasinduction,generalanesthesia
have been proposed to evaluate the degree of non-com- wascontinuedwithacontinuousi.v.infusionofRemifen-
paction. For instance, the ratio between non-compacted tanil. All anesthetic procedures were completed without
andcompacted myocardium distance (NC/C ratio)[2,4]; anyadverseeffects.
theX:YratiodescribedbyChinetal.[5],whereXrepre-
sents depth of the trabecular recesses and Y represents Diagnostic criteria
totalfree-wallthicknessatthepeakofthetrabeculations; For the diagnosis of LVNC, we used previously defined
andtheNCareaonatwochamberviewproposedbyYou- diagnosticcriteriaduringthewholestudy[2].Thesewere
sefetal.[12].Thesuggestionfromstudiesinadultsisthat as follows: 1) absence ofcoexisting cardiac anomalies; 2)
these measures of non-compaction may correlate with a presenceofmultipleprominenttrabeculations;3)presence
decrease of Ejection Fraction (EF). For instance, it has of multiple deep inter-trabecular recesses and 4) NC/C
been reported that EF is significantly decreased as the distanceratio>2.3,i.e.themaximalend-diastolicdistance
extentofnon-compactionincreases[13].Inchildrenhow- of the NC endocardial layer to the compaction myocar-
ever, there are only a few studies reporting the relation diumlayergreaterthan2.3.
betweenmorphologicalfeaturesofLVNCandventricular For this study we considered normal EF values > 53%
performance. Chin et al. [5] found that the echocardio- and indexed CMM values that were between percentiles
graphic ratio X to Y was greater in patients with LVNC 25and75[15]orwithintheconfidenceintervalofnormal
compared withcontrolsbutthisstudydidnot relate this values[16].
ratiotocardiacfunction.Pignatellietal.[14]reportedthat
asignificantnumberofchildrenwithLVNCcouldhavea CMR Protocol
deteriorated ventricular function window, followed by a CMRwasperformedona SiemensAvanto 1.5TMRsys-
periodoftransient recovery,andfinallyalater deteriora- tem(Siemens,Erlangen,Germany).Theprotocolincluded
tionstage.Anotherstudyperformedinchildrenandadults several MR imaging sequences; however, as described in
[11], found that in some patients, late gadolinium the next section, we only used some of these sequences
enhancement (LGE) can be observed not only in non forthepurposeofthisstudy.Inparticular,theMRproto-
compactedsegments but also in normal segments of the col included b-SSFP cine images in the horizontal long
heart. axis(4-chamber(4Ch)view),shortaxis(SA),verticalLong
In this study we proposetouse CMR to find if there is Axis(LA)viewsforventricularwallmotionanalysis.The
anycorrelationbetweenventricularperformancewithdif- SAviewincluded10-14slicescoveringbothventriclesfor
ferent morphological features, such as the Compacted theassessmentofcardiacfunction.Inallpatients,aMag-
Myocardial Mass (CMM), Compacted and Non-Com- netic Resonance Angiography (MRA) sequence was
pactedMyocardialAreas(CMA,NCMA),NC/Cratio,dis- obtained immediately after injection of a gadolinium-
tributionofLVNC,andregionalwallmotionabnormalities basedcontrastagent(0.2mmol/kgofgadopentetatedime-
inchildrenandyoungadolescentswithisolatedLVNC. glumine). The same protocol was applied in all patients
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withtheexceptionoftheLGEsequence.From2007,our ESV,CMM,wereobtainedbymanualsegmentationofthe
Institutionbegantoperformmyocardialviability;therefore ventricle and mass in the SA view. For calculating CMA
LGEwasperformedinonlysevenpatients.LGEconsisted and NCMA, manual segmentation was performed at the
ofaninversionrecoverysequenceacquiredbetween10to end-diastolic phase using LA and 4CH views. Previous
12minutes after the MRA sequence. The inversiontime studiesmeasuredthearea ofLVNContheLAview [12];
was chosen from a Look-Locker sequence [17] selecting however,thisviewonlyincludesthesuperiorandinferior
the time when blood was nulled. All LGE scans were walls. Since non-compaction can also affect the septum
acquired during mid diastole, the triggering time and andlateralwalls,wecalculatedthetotalCMAandNCMA
acquisitionwindowweresetforeach patientaccordingly as the sum of the areas measured on the LA and 4CH
to their heart rate. Additionally, a through-plane phase views. All measurements of volumes and areas were
contrast sequence at the level of the Atrio-Ventricular indexedtobodysurfacearea.
(AV) valves were acquired for flow quantification. The TheNC/CdistanceratiowasmeasuredintheSAorin
flow scans were acquired with high temporal resolution the 4CH view at the end-diastolic phase of the cardiac
(between 20 ms and 40 ms depending on the heart rate) cycleforoptimalvisualizationofthe2layersasproposed
toavoidanysummationoftheeandawaves.Asummary in[13].
of the acquisition parameters for these sequences are Tomeasure thedistribution of non-compaction, aseg-
depictedintable1. mentalanalysiswasevaluatedusingastandard17-segment
cardiacmodelasdefinedbytheAmericanHeartAssocia-
CMR measurements tion/AmericanCollegeofCardiology(AHA/ACC)forstan-
In allpatientssystolic ventricular functionwasevaluated dardized myocardial segmentation [18]. As previously
by measuring the End-Systolic Volume (ESV) and End- recommended, theapex(segment 17)wasexcludedfrom
DiastolicVolume(EDV).Subsequently,strokevolumeand the quantitative analysis [2]. Two blinded observers (L.C
EFwereobtainedfromthelasttwomeasurements. and S.U.) determined visually in the SA, 4CH and LA
Inordertoanalyzeiftherewasanycorrelationbetween viewsifanyparticularsegmentwasaffectedbynoncom-
ventricular performance andmorphologicalfeatures, dif- paction(reducedmyocardialmassandtrabeculations).
ferentmeasuresincludingCMM,CMAandNCMA,NC/ Left ventricular wall motion abnormalities were
Cratio,X:Yratio,distributionofleftventricularnon-com- assessed by visual inspection on the SA, 4CH and LA
paction, and assessment of ventricular wall motion views according to a five point score system as follows:
abnormalitieswereevaluated. 1: normal, 2: mild to moderate hypokinesia, 3: severe
All these morphological and functional measurements hypokinesia, 4: akinesia, and 5: dyskinesia.
were obtained by manual segmentation using commer- Measurements of the velocity of early to late diastolic
ciallyavailablesoftware(Argus,Siemenshealthcare).EDV, filling (E/A) were performed on flow images at the AV
Table 1Imaging Parameters ofCMRscans
Parameter Cineb-SSFP MRA Flow LGE
FOV(FH-AP-RL)(mm) 390×255 280×240×140 300×270 390×255
AcquiredResolution(mm) 2×2 2×2×2 2.1×2.1 1.5×1.5
ReconstructedResolution(mm) 1.6×1.6 1×1×1 1.15×1.15 1.5
Slicethickness(mm) 8 2 10 7
Nrofslices 1 110-180 1 3
TR/TE(ms) 2.9/1.5 3.8/1.9 4.7/2.6 3.2/1.6
TFEfactor 9-13 15-20 3-4 10
Grappa 2AP 2AP1-1.5RL no no
flipangle 60 60 15 15
Nrcardiacphases 20-30 2 20-40 1
TemporalResolution(ms) 30 60 28.2 70
Triggeringmodality Retrospective none Retrospective Gate
Averages 1 1 3 1
V (cm/s) NA NA 150-300 NA
enc
LGElategadoliniumenhancement
FHfeethead
APanteriorposterior
RLrightleft
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valves to find any suggestion of diastolic dysfunction. E/ more common in the apical level, present in all patients
A reversal was recorded as an indication of diastolic (100%), as compared with the mid-ventricular level
dysfunction. (80%) and the basal level (33%). In 26,6% of the patients
Linear regressions and correlations were calculated for the non-compaction involved all four segments at the
all the different morphological measurements versus apical level. At the mid-ventricular level, inferior and
ESV, EDV and EF. lateral segments were more commonly affected. No
patients had non-compaction in the septal wall at the
Results mid-ventricular or basal level. The mean number of
Clinical information included a summary of past medi- affected segments was 5.86 (range: 2-11). We found five
cal history. No patient had a family history of any cardi- patients with ≥ 9 segments affected with non-compac-
omyopathy. The most common clinical findings were tion. These patients were the only ones with non-com-
arrhythmias, present in five patients, with two patients paction also at the basal ventricular level (Figure 3).
having the Wolff-Parkinson-White syndrome. Three Figure 4 shows the images obtained in one patient with
patients complained of dyspnea, two of chest pain and severe isolated LVNC.
four patients of palpitations. Only one patient was Only five patients had wall motion abnormalities (Fig-
asymptomatic. ure 5). Interestingly, this figure shows that in the more
Table 2 and 3 summarizes the functional and morpho- severe cases, wall motion abnormalities were seen in
logical CMR findings. The mean EF was 51.7% (SD = some segments not affected with non-compaction, invol-
10.93%, and ranging from 28.4% to 64.2%) and only five ving especially some septal segments at the apex, mid
patients had a decreased EF (< 53%). The E/A ratio sug- and basal levels. As can be seen in Table 4 and Figure 3,
gested that four of them had diastolic dysfunction as those five patients with wall motion abnormalities were
well. There was only one patient with diastolic dysfunc- the only ones with a reduced EF < 53%, with more than
tion but normal EF. Another five patients had abnormal nine segments affected with non-compaction and four
CMM values (greater than percentile 97 according to of them had evidence of left ventricular diastolic
[15]), but none of them had decreased EF. LGE was not dysfunction.
present in patients (n = 7) who underwent the LGE
sequence. Discussion
Figure 1 shows the linear regression and correlations Several echocardiographic and CMR measurements have
between EF and the five morphologic measurements: been proposed to diagnose LVNC. There has been an
NC/C ratio, CMM, CMA, NCMA and the X:Y ratio. As association between adult patients with isolated LVNC
shown in this figure, the highest correlation was 0.64 for and depressed left ventricular function; however the rea-
the relation between NCMA and EF. Although the son for this remains unclear. In adults, it has been
mean NC/C ratio was relatively high (3.82 ranging from found that some of these measurements such as NC/C
2.57 to 5.05) and the X:Y ratio indicated the presence of ratio and NCMA are well correlated with systolic dys-
LVNC, there was a poor correlation between both mea- function. However, in children these relations have not
surements and EF. Better correlations were obtained been yet established.
when comparing CMM, CMA, and NCMA with ESV Inthisstudyweinvestigatedtherelationbetweendiffer-
and EDV (Figure 2). However, based on the linear ent anatomical measurements with ventricular perfor-
regression plots it is difficult to establish linear relation- mance using CMR on a pediatric population. We found
ships between any of these measurements. Distribution thatonlyfivepatientshadadecreasedEF(<53%)andthat
of segments with LVNC morphology is shown in table 4 there was a poor correlation between EF and measure-
and Figure 3. Non-compaction of the left ventricle was mentssuchasCMM,CMA,NC/CratioandtheX:Yratio
Table 2Summaryofthe analyzed functional andmorphological CMRparameters.
EF(%) EDV(cc/m2) ESV(cc/m2) CMM(g/m2) CMA(cm2/m2) NCMA(cm2/m2) NC/Cdistanceratio
Mean/SD 51.69/10.93 83.99/14.94 40.67/13.17 61.47/16.79 21.68/6.46 25.47/8.33 3,83/0,79
Range 28.40-64.20 60.10-111.80 27.40-80.00 44.50-105.40 15.95-40.22 15.91-49.09 2.57-5.05
EF,ejectionFraction
EDV,end-diastolicVolume
ESV,end-systolicvolume
CMM,compactedmyocardialmass
CMA,compactedmyocardialArea
NCMA,noncompactedmyocardialarea.
NC/C,non-compactiondividedbycompaction
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Table 3Description ofCMR morphological and cardiac function findings in children andyoung adolescent with
isolated LVNC.
Patient Agey. EF EF< Abnormal Diastolic LGE NC/C WallMotion NumbersofLVsegments
o % 50% CMM Dysfunction ratio Abnormalities withNC
1 10.4 58 No Yes No N/A 4,7 Nono 6
2 14.1 64.2 No No No N/A 4,6 No 3
3 3.4 44.6 Yes No Yes N/A 5 Yes 10(NCinbasalsegment)
4 17 53.5 No Yes No N/A 2,9 No 6
5 14.1 53.3 No No No Negative 3,7 No 4
6 8.9 61.6 No Yes No Negative 3,7 No 4
7 14.1 54.4 No No No N/A 3,7 No 6
8 11.5 63.4 No No No Negative 3,7 No 6
9 0.6 28.4 Yes No Yes Negative 4,7 Yes 10(NCinbasalsegment)
10 4.3 35.7 Yes No Yes N/A 5 Yes 11(NCinbasalsegment)
11 13.1 60.5 No Yes No Negative 3 No 4
12 11.2 53.6 No No Yes N/A 2,6 No 4
13 1.9 41.5 Yes No Yes N/A 3,6 Yes 12(NCinbasalsegment)
14 5.5 41.5 Yes No No Negative 3,6 Yes 11(NCinbasalsegment)
15 14.8 61.2 No No No Negative 3 Yes 6
CMM,compactedmyocardialMass
EF,ejectionFraction
LGE,Lategadoliniumenhancement
NC/C,non-compactiondividedbycompaction
LV,leftventricular
N/A,notapplicable
Figure 1 Linear regression and correlations between EF and different morphological measurements. There was a low correlation
betweenalltheseparametersandEF.Noneofthesemeasurementsareagoodindicatorofventricularperformanceinchildrenwithisolated
LVNC.(Compactedmassising/m2andmeasuresofareasareincm2/m2).
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Figure2Linearregressionandcorrelations.Linearregressionandcorrelationsbetweenmyocardialcompactedmass,noncompactedarea,
andcompactedareawithESVandEDVwerehigherthanthosefoundinforEF(Figure1).However,linearregressionplotswerealsoscattered
indicatingapoorlinearrelationshipbetweenthesemorphologicalindexesandESVorEDV.(LV-ESVandLV-EDVareincc/m2,compactedmassin
g/m2andmeasuresofareaareincm2/m2).
proposedbyChinetal.[5].Abettercorrelationwasfound becomes more overt with increasing age [14,19,20].
between EF and NCMA (R = 0.67). Yousef et al. [12] Recently, Pignatelli et al. [14] reported that nine patients
found a similar correlation but in an adult population, presenting depressed LV contractility in the first year of
with the NCMA being measured in a LA view. In our life and then had a transient recovery of LV function;
studytheNCMAwascalculatedasthesumoftheNCMA however, LV EF deteriorated later in life.
onLAand4Chviews;thiswasdonetoincludeanyseptal In our study, the severity and extension of non-com-
and lateral walls that may involve any degree of non- paction was quantified by calculating the number and
compaction. distribution of 16 myocardial segments that had some
We also found that the relation between the evaluated degree of non-compaction. According to the literature,
morphological indices with ventricular dysfunction was in adults, the distribution of left ventricular non-com-
poor in children with LVNC. There was a small associa- paction is mainly at the apical (all segments), mid-infer-
tion between the degree of non-compaction and ventri- ior and mid-lateral segments [4]. In our pediatric
cular dysfunction; however it was difficult to establish a population, the distribution of segments affected by
linear relationship between these parameters. This is
probably explained by the greater cardiac reserve pre-
sent in a younger population. However, it is important
to continue assessing ventricular function in these
patients along time, since it has been shown that this
variable could potentially deteriorate with age. All these
observations suggest that ventricular dysfunction
Table 4Distribution ofnon-compaction in different
segments atdifferent levels ofthe leftventricle.
Basal Med Apical Total
Anterior 2 6 13 21
Inferior 2 4 10 16
Septal NA NA 4 4
Lateral NA NA 15 15
Anteroseptal 0 0 NA 0
Inferoseptal 0 0 NA 0
Figure 3 Distribution of segments with LVNC in all patients.
Inferolateral 4 12 NA 16
Thosepatientswithmorethan9affectedsegmentsweretheonly
Anterolateral 4 12 NA 16 oneswithcompromiseofleftventricularperformance.Basal
Total 12 34 42 88 segmentinvolvementtendedtooccurinmoreseverecases.
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Figure4AfiveyearsoldfemalewithEF41.5%.Steady-statefreeprecessionmagneticresonanceimaginginaLongAxis(a),four-chamber
(b)andshort-axis(c,d,e)viewsdemonstrateextensivetrabeculationsoftheLVwallinthebasal(e),mid(d)andapicalsegments(c).
Figure5Resultofthe17-segmentanalysisofwallmotionabnormalityandnoncompation(numbersindicatedegreeofwallmotion
abnormalitiesaccordingtotext).ThesepatientweretheonlyoneswhohadwallmotionabnormalitiesandandEjectionFraction(EF)<53%.
Interestingly,inthemoreseverecasestherewerewallmotionabnormalitiesinseptalsegmentsnon-affectedbynocompaction.
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non-compaction was very similar to that found in Conclusions
adults. In conclusion, in children and young adolescents with
Additionally,wefoundthatifanybasalsegmentshowed isolated LVNC, none of the anatomical measurements
somedegreeofnon-compaction,regionalventricularcon- including CMM, CMA, NC/C ratio and the X:Y ratio
traction was abnormal causing a depression of systolic had a good correlation with cardiac function, only
and/ordiastolicfunction.Inparticular,wefoundthatfive NCMA had a better relation with EF but this was still
patientshadnineormoresegmentswithventricularnon- low. Nevertheless, the number of affected segments was
compaction, and all of them had at least one basal seg- a strong predictor of ventricular function. The segmen-
ment with non-compaction and presented regional wall tal pattern of involvement in childhood was similar to
motionabnormality.Importantly,thesame patientswere that found in previous adult studies. Furthermore, invol-
theonly ones witha decreasedEFandfour also had dia- vement of basal segments in our cohort was confined to
stolicdysfunction.Otherstudiesperformedinadults,have those with more extensive disease.
shown a similar relation between the extension of non-
compactionandtheseverityofthisdisease[12,13].Dodd
Acknowledgements
et al [13], showed that the extension of left ventricular
TheauthorsthanktheVicerrectoriadeInvestigaciónyDoctoradoat
non-compactionat the midventricular levelsorthe pre- PontificiaUniversidadCatolicadeChileandConicytforfundingsupport
sence of LGE patterns at basal levels, imply more severe (Fondecyt11100427andAnilloACT079).
clinical disease in an adult population. These studies are
Authordetails
onlinewithourfindings,whichindicatethattheseverity 1RadiologyDepartment,PontificiaUniversidadCatólicadeChile.Marcoleta
ofthe disease inchildren may be determinedwhennon- 367,HospitalClinicoUniversidadCatolica,Santiago8330024,SantiagoChile.
2DepartmentofElectricalEngineering,PontificiaUniversidadCatólicade
compactionextendtothebasalsegments.
Chile.Av.VicuñaMackenna4860,Macul7820436,Santiago,Chile.
LGE has been previously detected using CMR in 3BiomedicalImagingCenter,PontificiaUniversidadCatólicadeChile.Av.
patientswithLVNC.However,ourresultsfromCMRlate VicuñaMackenna4860,Macul7820436,Santiago,Chile.4PediatricCardiology
Department,PontificiaUniversidadCatólicadeChile.Marcoleta367,Hospital
gadolinium enhancement were negative for all patients.
ClinicoUniversidadCatolica,Santiago8330024,SantiagoChile.5Divisionof
Future studies may consider using stress perfusion with ImagingSciences,King’sCollegeLondon.TheRayneInstitute,4thFloor
CMR. This technique could potentially provide valuable LambethWingSt.Thomas’Hospital,LondonSE17EH,UK.
information about microcirculation in the myocardium
Authors’contributions
andperformanceoftheheart[13,20-22]. SUconceivethestudyandparticipatedinitsdesign,coordination,collection
One drawback of the study is the limited clinical andstatisticalanalysisofthedata,anddraftingofthemanuscript.
LCparticipatedinthedesignofthestudy,acquisitionandanalysisofthe
information available from patients. This information
data,anddraftingthemanuscript.
was difficult to obtain since most were outpatients GUparticipatedinthedesignofthestudyanddraftingthemanuscript.
referred from other institutions. We are currently plan- THparticipatedinanalysisofthedata,anddraftingthemanuscript.
RPparticipatedintheacquisitionandanalysisofthedata,anddraftingthe
ning to perform a follow up study of these patients to
manuscript.
analyze any possible relation between clinical symptoms FHparticipatedinthedesignofthestudyanddraftingthemanuscript.
and image findings. Another limitation is that the study MAparticipatedinthestatisticalanalysisofthedata,anddraftingthe
manuscript.
was a retrospective review of a relatively small number
CTparticipatedinthedesignthestudy,anddraftingofthemanuscript
of patients. However, to the best of our knowledge, this PIparticipatedinthedesignthestudy,anddraftingofthemanuscript
is the largest series of children and young adolescents Allauthorsreadandapprovedthefinalmanuscript.
with isolated LVNC assessed with CMR. Even though,
Competinginterests
we performed a retrospective review of the CMR Theauthorsdeclarethattheyhavenocompetinginterests.
images, the same CMR protocol, (with the exception of
Received:19July2011 Accepted:31January2012
LGE), was applied in all patients, providing consistent
Published:31January2012
data for assessment of morphology and cardiac function
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