Andersons pediatric cardiology 1368
Bạn đang xem bản rút gọn của tài liệu. Xem và tải ngay bản đầy đủ của tài liệu tại đây (105.87 KB, 3 trang )
Definitions
Thewordtumorisusedinthischaptertorefertoaneoplasm,oranabnormal
growthofcells.Thisdifferentiatesitfromothertissuemassessuchas
hematomas,thrombi,orvegetations.
▪Benigntumors,asopposedtomalignanttumors,do
notinvadeadjacenttissuesanddonotmetastasizeto
distantsites.
▪Primary:locatedatsiteoftissueorigin.
▪Secondary:foundatdistantsitesduetometastasisor
directinvasion.
▪Hamartomas:benign,characterizedbyovergrowth
ofnormaltissuewithfewatypicalchanges,andthe
inabilitytoundergomitosisandmetastasis.1
▪Polypoid,papillary,andvillous:refertothegross
structure,resemblingpolyps,papillae,orvilli,
respectively.
▪Sessile:attachedtoadjacenttissuebyabroadbase.
▪Pedunculated:attachedbyapeduncle,orstalk.
▪Circumscribed:confinedtoalimitedarea.
▪Encapsulated:surroundedbyarimofconnective
tissue.
ClinicalSignsandSymptoms
Althoughcardiactumorsareusuallybenigninchildren,theymayinducelifethreateningsymptoms.Theirclinicalmanifestationsareoftennonspecific.They
maymimicmanyotherdiseasesoftheheartandlungs.Classically,theclinical
presentationisdividedaccordingtowhetheritistheconsequenceofsystemic,
embolic,orcardiaceffects(Table52.4).
Table52.4
ClinicalManifestationsofCardiacTumors
Symptoms/Signs
SYSTEMIC
Fever
General
malaise
Weightloss
Fatigue
Digital
clubbing
Raynaud
phenomenon
Myalgiaand
arthralgia
EMBOLIC
Systemic
embolism
Pulmonary
embolism
CARDIAC
Arrhythmias
Cardiacfailure
Pericardial
effusionand
tamponade
Obstruction
Tumorplop
Remarks
Maymimicendocarditisormalignantdisease
Maymimiccollagen,rheumatic,ormalignantdisease
Mayaffectalmostanyorgan
Causedbyembolizationofleft-sidedtumorfragmentsorthrombiaggregatedatitssurface
Causedbyembolizationofright-sidedtumors
Almostanyarrhythmiascanoccur
Completeatrioventricularblockandsuddendeathareseenastheextreme
Maymimicdilated,restrictive,orhypertrophicobstructivecardiomyopathy
Maypresentwithcardiomegaly,respiratorydistress,hydrops,pulmonaryedema,cyanosis
Seenmainlyinteratoma,malignanttumors,andsecondarylesions
Maycauseoutflowtractoratrioventricularvalveobstructionandmimicvalvardisease
Maypresentwithnewmurmur,syncope,suddendeath,oracutepulmonaryedema
Symptomsandmurmurmayrelatetothepatient'sposition
Isalmosttheonlyspecificsign(withtheposturalvariationofamurmur)
Resultsfromsuddentensiononthestalkofthetumorasitprolapsesduringdiastoleinto
theleftventricularcavitywiththepossibleimpactofthemassontheventricularwall
SystemicManifestations
Thesystemicmanifestationsoftumorsoftheheartaremanifoldandinclude
findingssuchasfever,generalmalaise,lossofweight,andfatigue.Digital
clubbing,Raynaudphenomenon,myalgia,andarthralgiaareusuallyassociated
withmyxomas.Allthesefeaturescanmimicinfectiveendocarditis,collagen
vasculardisease,rheumaticheartdisease,ormalignancy.11Theconstitutional
symptomsdisappearwhenthetumorisremoved.11Thereisevidencetosuggest
thattheinflammatoryandautoimmunemanifestationsseeninthepresenceof
myxomasmaybetheresultoftheproductionandreleaseofthecytokine
interleukin-6bythetumor.11–14
EmbolicManifestations
Embolicsignsarenotanuncommonmanifestationofcardiactumors,being
reportedin30%ofpatientswithleftatrialmyxomas.15Embolismisalsowell
describedinchildren16butisinfrequentduringfetallifeortheneonatalperiod.1
Therecanbeembolizationofeitherfragmentsofthetumoritself(Fig.52.1),or
thrombusaggregatedatthesurfaceofthetumor(Fig.52.2).Embolizationofthe
entiretumorhasbeendescribedinrarecases,oftenwithtragicconsequences.17
Thedistributionofsuchembolidependslargelyonthelocalizationofthe
primarytumoritself,alongwiththepresenceorabsenceofadditionalcardiac
malformations,suchasshuntsorabnormalflowpatterns.Embolizationoftumor
fragmentsoccursonlywhenthetumoritselfhasanintracavitaryextension.
However,thromboembolicformationcanoccuralsowithprimaryintramural
tumorsthatcompromisetheendocardiumofthecardiacchamber,eitherby
mechanicalcompressionorbyinducingafunctionaldisturbance.
