Andersons pediatric cardiology 1370
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FIG.52.3 Heartshownfromtheleftside.Ahugerhabdomyomahas
grownintheanterosuperiorwalloftheleftventricle,compromisingthe
ventricularcavityandproducingcardiacfailure.
Obstruction
Primarycardiactumorswithintracavitaryextensionmaycauseobstructionto
inflowandoutflowtracts,systemicandpulmonaryveinsandarteries,coronary
arteries,andinterferewithvalvarfunction(Fig.52.4A).Thesignsand
symptomsarethusmyriadanddependentonthechamberinvolvedandthesize
andnatureofthetumor.Theycanmimicvalvardisease,left-andright-sided
heartfailure,coronaryischemia,andcardiactamponade.Alltheseobstructive
manifestationsmayhaveasuddenonset,suchasinthecaseofapedunculated
highlymobileleftatrialtumor,whichcancausesyncope,suddenunexpected
death,oracutepulmonaryedema.1,3Suchfindingsareoftenintermittentand
mayrelatetothepostureofthepatient.Asstatedbefore,thepicturemaybe
furthercomplicatedbypulmonaryembolismandsecondarypulmonary
hypertension.Infetuses,obstructionisnotedtohappenatanystageofgestation,
andobstructionstosystemicvenousreturnand/orbothoutflowtracts
simultaneouslyarenotedtobeparticularlyworrisome.28
FIG.52.4 Rhabdomyoma(asterisk)oftheleftventricularoutflowtractwith
intracavitaryextensionin(A)diastolicand(B)systolicframes.Notecolor
flowturbulenceduringsystole.Thispatientwasmanagedconservatively,
withresolutionofoutflowtractobstructionasthetumorregressed.
PhysicalExamination
Ingeneral,physicalexaminationisnonspecific.Murmurs,whenpresent,arein
themselvesnonspecific,butcertainatypicalfindingsmaysuggestacardiac
tumorratherthanprimaryvalvarormyocardialdisease.Suchfindingsinclude
posturalvariationintheintensityofthemurmur,andthepresenceofaso-called
tumor“plop.”Theplophasbeendescribedparticularlyinpatientswithleftatrial
myxoma,beingcharacteristicofapedunculatedandhighlymobiletumor.This
plopwasheardinonlyapproximately16%ofthepatientsinonelargeseries.15It
mostlikelyresultsfromsuddentensiononthestalkofthetumorasitprolapses
duringdiastoleintotheleftventricularcavity,alongwiththepossibleimpactof
themassontheventricularwall.29
Dermatologicfindingscanalsoalertthecliniciantocertaintypesoftumors.
Tuberoussclerosiscomplex(TSC)isassociatedwithrhabdomyomasand
characteristicskinlesionssuchasfacialangiofibromas,hypomelanoticmacules,
shagreenpatches,andperiungualfibromas.30Carneycomplexisassociatedwith
atrialmyxomas.Thesepatientshaveavarietyofskinpigmentation
abnormalities,includingintensefrecklingandbluenevuses.31Gorlinsyndrome
isassociatedwithfibromasandischaracterizedbymultiplebasalcell
carcinomas.1,31
