Andersons pediatric cardiology 678
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IsomerismoftheAtrialAppendages
RohitS.Loomba,DianeE.Spicer,LaurenE.Raczka,RobertH.Anderson
Abstract
Isomerism,orso-calledheterotaxy,isamultiorganentitythathasanatomic
andfunctionalconsequences.Inthethorax,thereismirrorimageryofthe
usuallylateralizedorgansystems.Intheheart,themirrorimageryisseenin
theatrialappendages,whichcanbeofeitherleftorrightmorphology.
Cliniciansneedtorecognizeisomerismandtobeawareofitsassociated
findingsbecausetheyimpactbothmorbidityandmortality.Asmany
childrenwithisomerismwillrequirecardiacsurgery,thisrecognitionis
veryimportantforcardiologists.Patientswithisomerismmayhave
anomaliesofthenervous,sinopulmonary,pulmonary,cardiac,
gastrointestinal,genitourinary,hematologic,immune,andreproductive
systems.
Thegeneticunderpinningsofisomerismarestillbeingclarified,with
multiplegeneshavingbeenimplicated.Manyofthesegenesalsoplaya
roleinciliaryfunction.Thisisofparticularinterest,asmanyofthosewith
isomerismalsohaveciliarydysfunction.Thusthegeneticsandembryology
ofisomerismmayberelatedtothoseofciliarydysfunction.
Childrenwithisomerismwhohavefunctionallyuniventricularcongenital
heartdiseasehavedecreasedsurvivalcomparedtothosewiththesame
congenitalheartdiseasebutwithoutisomerism.Decreasingthisdifference
dependsonthoroughknowledgeoftheanatomicandfunctional
consequencesof,theinheritanceof,andsymptomaticcareof,isomerism.
KeyWords
Isomerism;heterotaxy;asplenia;polysplenia;functionallyuniventricular;
Kawashima;Fontan;Glenn;ciliarydyskinesia
