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Andersons pediatric cardiology 1319

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ArteriovenousCommunications
SrinathT.Gowda,AtharM.Qureshi,HenriJustino


Abstract
Thischapterdiscussesavarietyofarteriovenouscommunications,with
specialemphasisonthosethatresultincardiovascularconsequences,such
asvolumeand/orpressureoverloadofaspecificvascularbed,and
cyanosis.Wedistinguishbetweenacquiredneoplasmscomposedof
endothelialcells(hemangiomas)fromprimitiveerrorsofvascular
development(arteriovenousmalformationsandarteriovenousfistulas).

Keywords
Hemangioma;Arteriovenousmalformation;Arteriovenousfistula;
Arterioportalfistula;VeinofGalenmalformation;Coronaryfistula;
Pulmonaryarteriovenousmalformation;Osler-Weber-Rendusyndrome;
Hereditaryhemorrhagictelangiectasia;Cyanosis;Portalhypertension;
Pulmonaryhypertension
Therehasbeendramaticprogressinthefieldofvascularanomaliesinrecent
years,includingimprovedunderstandingoftheclassificationoftheselesions,
theirnaturalhistory,andoptimalmanagementstrategies.1Thissectiondiscusses
onlyvascularanomaliesthathavethepotentialtoposecardiovascular
ramifications,eitherduetoabundantarteriovenousshuntingpresentingwith
congestiveheartfailure(CHF)orpulmonaryhypertensionorduetoright-to-left
shunting(attheintracardiacorintrapulmonarylevel)presentingwithcyanosis.
Becauseofthehighlyvariedandimpreciseterminologiesusedinthepastto
describeahostofvascularanomalies,olderpublicationsaredifficulttorelyon
foraccuratediagnosesandarepredominantlyusefultoprovidehistoricalcontext
regardingtheearlydescriptionsofsuchconditions.Oneoftheearliestreportsof
“congenitalhemangioendothelioma”(atermthatshouldnolongerbeused
becauseitdoesnotconveywithclaritytheprecisenatureofthelesionaccording


tomodernstandards)withcardiovascularsymptomsininfancywasby
Kunstadterin1933,inwhichhedescribesa5-month-oldinfantwithdyspnea
andcyanosis.2LevickandRubie3describedasimilarcaseof
“hemangioendothelioma”simulatingcongenitalheartdiseaseinaninfantwith
dyspneaandcyanosis;pathologicfindingsshowedmultiplenodulesmeasuring
approximately1cmdistributedthroughoutbothlobesoftheliver;theheartwas


considerablyenlargedwithbiventricularhypertrophy,andaninteratrial
communicationwasseentogetherwithatinyarterialduct.Right-to-left
interatrialshuntingispresumedtohavebeenthecauseofcyanosis.Withthis
historicalcontextinmind,wewilldiscussspecificvascularanomaliesaccording
tocurrentlyacceptedterminology.1



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