Andersons pediatric cardiology 1305
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FIG.49.2 Neonatewiththeheartexteriorizedthroughthethoracicwall.
Notethelackofanypericardialcovering.Inthisinstance,theheartwas
safelyplacedwithinthethoraciccavity.(CourtesyDr.MarshallJacobs,
TempleUniversity,Philadelphia,PA.)
Thecasesmakingupthecombinedthoracoabdominalsubsetdifferfromthose
withexclusivelythoracicexteriorizationinthattheheartisbettercoveredbythe
bodywall,havingatleastacoveringofskinormembrane(Fig.49.3).These
patientsallfallwithinthesyndromeunifiedbyfiveanomalies,whichisusually
knownasthepentalogyofCantrell.Theanomaliesareamidlinedeficiencyof
theabdominalwall,adefectofthelowerpartofthesternum,adeficiencyofthe
pericardialsac,adeficiencyofthediaphragm,andanintracardiaccongenital
lesion.12Notallpatientswithextrathoracicheartsextendingintotheabdomen
haveallofthesefeatures.Indeed,thecasescanthemselvesbegroupedaccording
tothenumberofthefivefeaturesthatarepresent.13Lesserformsofthe
pentalogyincludethemidlinedeficienciesshowninFig.49.3,alongwith
protrusionsofventriculardiverticulumsthroughmidlinedeficienciesofthebody
wall(Fig.49.4).Completeexteriorizationoftheheartrepresentstheextreme
formofthesyndrome.Treatmentofthepatientswiththeabdominothoracictype
ofexteriorizationhaspreviouslyprovedmoresuccessfulthanforthosehaving
exclusivelythoracicexteriorization,althoughthepatientwiththeexteriorized
thoracicheartshowninFig.49.2wastreatedsuccessfully.Nonetheless,until
recently,veryfewpatientssurvivedreparativesurgery,althoughseveralpeople
withabdominalheartswhodidnotundergosurgeryhavebeendescribedas
survivingintoadultlife.Thesurgicalproblemsencounteredinrestoringthe
hearttothebodyareconsiderable,includingthesmallsizeofthedeficient
thoraciccavity,theexcessivelengthofthevenousandarterialconnectionstothe
extrathoracicheart,andthefrequentcoexistenceofalargeomphalocele.
Thereforetheprognosisforthosebornwithexteriorizedheartsisnowmarkedly
improved,albeitconditionedbytheseverityoftheassociatedintracardiac
defects.14
FIG.49.3 (A)Anteriorwallofthethoraxandabdomeninapatientwiththe
midlinedeficienciesofthepentalogyofCantrell.Theskinisintact,asisthe
pericardialcavity.(B)Openingthepericardialcavityrevealedamidline
heartwithitsapexalsointhemidline.Therewasisomerismoftheright
atrialappendagesandtheintracardiacanatomyoftetralogyofFallot.
(CourtesyDr.BensonR.Wilcox,UniversityofNorthCarolina,Chapel
Hill.)
FIG.49.4 Magneticresonanceimageshowingaleftventricular(LV)
diverticulumprotrudingthroughamidlinedeficiencyofthebodywall
(arrow).AO,Aorta;L,left;R,right;RV,rightventricle.(CourtesyDrWalter
Duncan,UniversityofBritishColumbia,Vancouver.)
