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SinusVenosusAtrialSeptalDefects:Background,Pathophysiology,Etiology

SinusVenosusAtrialSeptalDefects
Author:GaryMSatou,MD,FASEChiefEditor:HowardSWeber,MDmore...
Updated:Sep10,2015

Background
Insimpleterms,anatrialseptaldefect(ASD)isadeficiencyoftheatrialseptum.
Atrialseptaldefectsaccountforabout1015%ofallcongenitalcardiacanomalies
andarethemostcommoncongenitalcardiaclesionpresentinginchildrenand
adults. [1]Sinusvenosusatrialseptaldefectsaccountforonly10%ofatrialseptal
defects.Theremainingatrialseptaldefectsareostiumsecundumtype(70%),
ostiumprimumtype(20%),andunroofedcoronarysinus,orcoronarysinusseptal
defects,(<1%).Mostchildrenwithsinusvenosusatrialseptaldefectsare
asymptomaticbutmaydevelopsymptomsastheyage.
Excellentsurgicalresultswithamortalityratenear0%canbeexpected.Thisis
particularlytrueinpatientswhoundergorepairwhenyoungerthan15years.An
atrialseptaldefectwasthefirstlesionrepairedusingcardiopulmonarybypassin
1954byJohnGibbon,MD,attheMayoClinic.

Pathophysiology
Themorecommonsinusvenosustypedefect(oftenreferredtoasthe"usualtype")
occursintheupperatrialseptumandiscontiguouswiththesuperiorvenacava
(SVC).Thelesionisrostralandposteriortothefossaovalis(wheresecundumtype
defectsoccur)andisseparatefromit.Itisalmostalwaysassociatedwith
anomalouspulmonaryvenousdrainageoftherightupperpulmonaryveinintothe
SVC.Seetheimagebelow.

PanelA.Transesophagealechocardiogram(transverseview)ofapatientwithasinusvenosus
defectofthesuperiorvenacava(SVC)type.Theoriginaldefect(whitestarburst)hasbeen
repairedbyplacingabaffle(arrows),whichdirectsbloodfromtheanomalouslyconnectedright
upperpulmonaryveinintotheleftatrium(LA).Inthispatient,thebafflewasredundantsoata
morerostrallevel(PanelB),itcouldbeseen(blackopenarrows)tobulgeintothesuperiorvena
cava(SVC)rightatrial(RA)junction(trioofwhitearrows).Theremainderoftheatrialseptumis
denotedbytheduoofwhiteopenarrows.PanelCisatransesophagealechocardiogram,sagittal
view.Dopplercolorflowmappingverifiesthattheprotrudingbaffle(whiteclosedarrows)results
inanarrowingofthepathwayfromtheSVCtotheRA.Thequartetofwhiteopenarrowspointsto
theremainderoftheatrialseptum.

Lesscommonly,thedefectmayoccuratthejunctionoftherightatriumandinferior
venacava(IVC)andbeassociatedwithanomalousconnectionoftherightlower
pulmonaryveintotheIVC.Rarely,sinusvenosusdefectsoccurposteriortothe
fossaovaliswithoutborderingtheSVCorIVC.Thepredominanthemodynamic
consequenceisalefttorightshuntthroughthedefect.Seetheimagebelow.

PanelAisatransesophagealechocardiogram,transverseview.Thewhitestarburstshowsthe
sinusvenosusdefectoftheinferiorvenacava(IVC)type,lyingadjacenttotheIVCjunctionwith
therightatrium(RA).Theremainderoftheatrialseptumisjustoutoftheviewofthissectorbut
isrepresentedbythewhiteopenarrowheads.Theleafletsoftheclosedtricuspidvalve(TV)are

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SinusVenosusAtrialSeptalDefects:Background,Pathophysiology,Etiology

visible.RV=rightventricle.PanelBisatransesophagealechocardiogram,sagittalview.This
isthesamepatientasinPanelA.Thisviewprovesthattherostralportionoftheatrialseptum
(whichwouldbemissinginapatientwithasinusvenosusdefectoftheSVCtype)isintact.ct=
cristaterminalissvc=superiorvenacava.

Etiology
Duringnormalembryonicdevelopment,therighthornofthesinusvenosus
encompassestherightsuperiorvenacava(SVC)andinferiorvenacava(IVC).If
abnormalresorptionofthesinusvenosusoccurs,anatrialseptaldefectresultsnear
theorificeofeithertheSVCorIVC.
Atrialseptaldefectsoccurasassociatedanomaliesinmanymajorcomplex
congenitallesionsbutsinusvenosusatrialseptaldefectsoccurmoreoftenasan
isolatedabnormality.
Otherabnormalitiesmayexacerbateanatrialseptaldefect.Forexample,systemic
hypertensioninanadultwithasinusvenosusatrialseptaldefectmayresultinleft
ventricularhypertrophyandreduceleftventricularcompliance,which,inturn,
exacerbatestheatriallevellefttorightshunt.Mitralstenosis,whichiseither
congenitaloracquired,mayalsoexacerbatetheatriallevellefttorightshunt.

Epidemiology
UnitedStatesdata
Sinusvenosusatrialseptaldefectsrepresentapproximately1%ofcongenital
cardiaclesions.

Race,sex,andagerelateddemographics
Noracialpredilectionisknownhowever,atrialseptaldefectsaffectfemalesmore
oftenthanmales.Thefemaletomaleratiois2:1.Nodifferenceinoutcomeis
associatedwithsex.
Sinusvenosusatrialseptaldefectsarecongenitallesionspresentatbirth.Theage
atpresentationdependsonthesizeofthelefttorightshunt(atrialandpartial
anomalouspulmonaryvenousreturn).Atrialseptaldefectsininfancyareusually
asymptomatic.Theyareusuallydetectedbyechocardiographywhileundergoinga
cardiacevaluation.

Prognosis
Theprognosisisexcellentforyoungpatientswhoundergorepairofuncomplicated
defects.Repairdelayeduntilthethirddecadeoflifeisassociatedwithadecreasein
lifeexpectancy. [9]

Mortality/Morbidity
Surgicalrepairinthefirst2decadesoflifeisassociatedwithamortalityratenear
zero.Lifeexpectancyapproachesthatofthegeneralpopulationifthedefectis
repairedduringthistime.Rightheartdilationrapidlyregressesaftersurgery,and
thefunctionalresultisexcellent.Incasesofrepairduringadulthood,lifeexpectancy
maybedecreaseddespitesuccessfulrepair.Surgicalmorbidityratesarerelatedto
earlypostoperativepericardialeffusion,earlypostoperativepulmonaryvenousor
systemicvenousobstruction,andsupraventriculararrhythmias.Ifthebaffledirecting
pulmonaryvenousbloodtotheleftatriumisnotplacedcorrectly,itmayobstruct
pulmonaryvenousdrainage.IfthebafflebulgesintotheSVC,itmayobstructSVC
inflow,necessitatingtheplacementofanaugmentationpatchontheanterior
surfaceoftheSVCandrightatrialjunction.
Untreatedatrialseptaldefectsareassociatedwithasignificantlyshortenedlife
expectancy.Afterage20years,themortalityrateisapproximately5%perdecade
with90%ofpatientsdeadbyage60years.Thesepatientspresentwithanincrease
inlefttorightshuntingandoccasionallywithcongestiveheartfailurewithpulmonary
hypertensioninthefourthtosixthdecadesoflife.Guidelinesforthediagnosisand
treatmentofpulmonaryarteryhypertensionhavebeenestablished. [2]Late
problemsinuntreatedpatientsalsoincludetheriskofparadoxicalembolusaswell
asatrialfibrillation,pulmonaryhypertension,andrightheartfailure.

Complications
Complicationsincludethefollowing:
Sinusnodedysfunction
Pulmonaryvenousobstruction
Atrialfibrillation,atrialflutter,orsupraventriculartachycardia(SVT)
Pulmonaryhypertension
Atrialbaffleleak
Pericardialeffusionorpostpericardiotomysyndrome
SVCsyndrome

PatientEducation
Patienteducationmainlyfocusesonpreoperativeandpostoperativecareand
recovery,whichareespeciallyimportantinyoungchildrenundergoingsurgery.
Centerswithexperiencedchildlifepersonnelareinvaluableinpreparingchildrenfor
openheartsurgery.
ClinicalPresentation

ContributorInformationandDisclosures
Author

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SinusVenosusAtrialSeptalDefects:Background,Pathophysiology,Etiology

GaryMSatou,MD,FASEDirector,PediatricEchocardiography,CoDirector,FetalCardiologyProgram,Mattel
Children'sHospitalAssociateClinicalProfessor,DepartmentofPediatrics,UniversityofCalifornia,LosAngeles,
DavidGeffenSchoolofMedicine
GaryMSatou,MD,FASEisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,
SocietyofPediatricEchocardiography,AmericanCollegeofCardiology,AmericanHeartAssociation,American
SocietyofEchocardiography
Disclosure:Nothingtodisclose.
Coauthor(s)
BrianLReemtsen,MDAssistantProfessorofCardiothoracicSurgery,KeckSchoolofMedicine,Universityof
SouthernCalifornia
BrianLReemtsen,MDisamemberofthefollowingmedicalsocieties:AmericanMedicalAssociation,Societyof
ThoracicSurgeons,WesternThoracicSurgicalAssociation
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
MaryLWindle,PharmDAdjunctAssociateProfessor,UniversityofNebraskaMedicalCenterCollegeof
PharmacyEditorinChief,MedscapeDrugReference
Disclosure:Nothingtodisclose.
AlvinJChin,MDEmeritusProfessorofPediatrics,UniversityofPennsylvaniaSchoolofMedicine
AlvinJChin,MDisamemberofthefollowingmedicalsocieties:AmericanAssociationfortheAdvancementof
Science,SocietyforDevelopmentalBiology,AmericanHeartAssociation
Disclosure:Nothingtodisclose.
ChiefEditor
HowardSWeber,MDFSCAI,ProfessorofPediatrics,SectionofPediatricCardiology,PennsylvaniaState
UniversityCollegeofMedicineDirectorofInterventionalPediatricCardiology,PennStateHersheyChildren's
Hospital
HowardSWeber,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,
AmericanCollegeofCardiology,SocietyforCardiovascularAngiographyandInterventions
Disclosure:Nothingtodisclose.
AdditionalContributors
CharlesIBerul,MDProfessorofPediatricsandIntegrativeSystemsBiology,GeorgeWashingtonUniversity
SchoolofMedicineChief,DivisionofCardiology,Children'sNationalMedicalCenter
CharlesIBerul,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,Heart
RhythmSociety,CardiacElectrophysiologySociety,PediatricandCongenitalElectrophysiologySociety,
AmericanCollegeofCardiology,AmericanHeartAssociation,SocietyforPediatricResearch
Disclosure:Receivedgrant/researchfundsfromMedtronicforconsulting.
Acknowledgements
TheauthorsandeditorsofeMedicinegratefullyacknowledgethecontributionsofpreviousauthorsJeffLMyers,
MD,PhD,andJamesJaggers,MD,tothewritinganddevelopmentofthisarticle.

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