Andersons pediatric cardiology 1315
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topsy-turvyarrangement.(A)Theventricles(RV,LV)arelocated
superoinferiorlybutwithoutanyrotationaltwisteddeformity.(B)Thearterial
trunks(aorta[Ao],pulmonaryartery[PA],rightpulmonaryartery[RPA]),are
displaceddownwards,withthebrachiocephalicarteriesandthecavalveins
elongatedastheyextendtothemediastinum(seeFig.49.18).LA,Left
atrium;RA,rightatrium.
FIG.49.24 Magneticresonanceimageshowingtheelongated
brachiocephalicarteriesproducedinconsequenceofthetopsy-turvy
arrangementoftheheart.Ao,Aorta.
FIG.49.25 Reconstructedcomputerizedtomographicimagefromthe
patientshowninFig.49.21illustratingtheelongationofthetracheaand
narrowingoftheleftbronchus(asterisk)asaconsequenceofthelow-lying
aorticarchandarterialductproducedbythetopsy-turvyarrangement.
AnnotatedReferences
KanagasuntheramR,VerzinJA.Ectopiacordisin
man.Thorax.1962;17:159–167.
Anextensivereviewofthecasesdescribedatthe
timeofwriting,showingthevariedpatternsfor
exteriorizationoftheheart..
CantrellJR,HallerJA,RavitchMM.Asyndrome
ofcongenitaldefectsinvolvingtheabdominal
wallsternum,diaphragm,pericardiumandheart.
SurgGynecolObstet.1958;107:602–614.
Theinitialdescriptionoftheconstellationof
lesions,nowknownasthepentalogyofCantrell,
theextremeformsofthesyndromebeing
exteriorizationoftheheart,butwithlesserforms
showingventriculardiverticulaextending
throughthemidlinedefectsofthebodywall..
ToyamaWM.Combinedcongenitaldefectsofthe
anteriorabdominalwall,sternum,diaphragm,
pericardiumandheart:acasereportandreview
ofthesyndrome.Pediatrics.1972;50:788–792.
Aneruditereview,albeitbasedonacasereport,
showingthatallfiveofthefeaturesinitially
describedbyCantrellandhiscolleaguesarenot
presentwhentherearemidlinedeficienciesof
thebodywall..
