Andersons pediatric cardiology 695
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theheterotaxysyndromeswithasplenia,or
polysplenia,ornormallyformedspleen:an
erroneousconcept.AmJCardiol.
1990;66:1504–1506.
Thismanuscriptprovidesarebuttaltothenotionof
atrialisomerismandmakesthepointthatthe
atriumsthemselvesarenotisomeric.Thishelped
developtheconceptofisomerismoftheatrial
appendagesandnottheatriumsthemselves..
UemuraH,HoSY,AndersonRH,etal.The
surgicalanatomyofcoronaryvenousreturnin
heartswithisomericatrialappendages.JThorac
CardiovascSurg.1995;110:436–444.
Thisnecroscopystudycharacterizesthecoronary
venousreturninheartswithisomerism.Of
particularnote,thestudyconcernsthedirect
drainageofsmallercoronaryveinsintothe
posterioraspectoftheatrium..
UemuraH,HoSY,AndersonRH,YagiharaT.
Ventricularmorphologyandcoronaryarterial
anatomyinheartswithisometricatrial
appendages.AnnThoracSurg.1999;67:1403–
1411.
Thisnecroscopystudycharacterizestheventricular
morphologyandcoronaryarterialbranching
patternsinthosewithisomerism,highlighting
thedifferencesbetweenthosewithrightandleft
isomerism.Thestudydemonstratesthatnota
trivialproportionofpatientswithisomerismwill
haveabnormalcoronarybranching..
SmithA,HoSY,AndersonRH,etal.Thediverse
cardiacmorphologyseeninheartswith
isomerismoftheatrialappendageswith
referencetothedispositionofthespecialised
conductionsystem.CardiolYoung.
2006;16:437–454.
Thisnecroscopystudyinvolveddelineationofthe
cardiacconductionsysteminheartswith
isomerism.Thepresenceofmultipleatrial
pacemakersandatrioventricularnodeswas
described.Alsoimportantly,thelocationofthe
multipleatrioventricularnodeswasdescribedin
thesettingofvariouscardiacmorphology..
LoombaRS,GeddesGC,BaselD,etal.
Bacteremiainpatientswithheterotaxy:areview
andimplicationsformanagement.Congenit
HeartDis.2016;11(6):537–547.
Thisreviewhighlightsdataregardingtheriskof
bacteremiainthosewithisomerismandalso
providesevidence-basedguidelinesonhowto
evaluateisomerismpatientsforriskof
bacteremiaandhowtobestminimizethisrisk..
NakhlehN,FrancisR,GieseRA,etal.High
prevalenceofrespiratoryciliarydysfunctionin
congenitalheartdiseasepatientswithheterotaxy.
Circulation.2012;125:2232–2242.
Thisstudycharacterizestheresultsofciliary
videomicroscopyperformedonnasalepithelial
cellsobtainedvianasalswabsfrompatientswith
andwithoutisomerism.Exhalednasalnitric
oxidelevelsinthesepatientsarealsoquantified.
Thisstudydemonstratesalargeburdenofciliary
dysfunctioninthosewithisomerismand
demonstratesthatthosewithisomerismmayalso
havelowerexhalednitricoxidelevels..
LoombaRF,AndersonRH.Geneticdisturbancesin
patientswithbodilyisomerismfromasingle
center:clinicalimplicationsofaffectedgenes
andpotentialimpactofciliarydyskinesia.
Cardiogenetics.2016;6:15–21.
Thisstudycharacterizestheresultsofgenetic
microarraytestinganddemonstratesthat
particularaffectedgenestendtoproducesimilar
phenotypes.Thestudynotonlydescribesthe
cardiacphenotypebutalsoarguesthatperhaps
theclinicalhospitalcourseispartofthe
phenotyperesultingfromspecificgenetic
perturbations..
