Ventricular Function and Blood Flow in Congenital Heart Disease

386 Pages·2005·6.58 MB·English

Ventricular
Functionand
BloodFlowin
CongenitalHeart
Disease
P1:FAW
BY042-FM BY042-Fogel January10,2005 20:38 CharCount=0
TomywifeRani,withoutwhoseloveandsupport
thisbookwouldnotbepossibleandwillalwaysbe
thebetterpartofme
To my children Arielle and Benjamin, for always
keepingmeyoungandenergetic
TomybrotherBobby,whotaughtmethatbeinga
trueseekeroftruthandunderstandingisnotjusta
dreamand
TomymotherSylvia,whohelpedmebecomewhoI
amtoday
ii
Ventricular
Function and
Blood Flow in
Congenital Heart
Disease
EDITED BY
Mark A. Fogel,
MD, FACC, FAAP
AssociateProfessorofPediatricsandRadiology
DirectorofCardiacMRI
TheChildren’sHospitalofPhiladelphia
TheUniversityofPennsylvaniaSchoolofMedicinePhiladelphia,Pennsylvania
(cid:2)C 2005byBlackwellPublishing
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Firstpublished2005
ISBN-13:978-1-4051-2211-5
ISBN-10:1-4051-2211-0
LibraryofCongressCataloging-in-PublicationData
Ventricularfunctionandbloodflowincongenitalheartdisease/editedbyMarkA.Fogel.
p.;cm.
Includesbibliographicalreferencesandindex.
ISBN-13:978-1-4051-2211-5
ISBN-10:1-4051-2211-0
1.Congenitalheartdiseaseinchildren–Pathophysiology. 2.Heart–Ventricles.
[DNLM:1.HeartDefects,Congenital. 2.VentricularFunction.WG220V46652005]
I.Fogel,MarkA.
RJ426.C64V462005
618.92(cid:3)12043–dc22
2004018315
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Contents
ListofContributors,vii 9 NuclearCardiologyandPositronEmission
Tomography,147
Foreword,ix
BruceY.Lee&MartinCharron
Preface,x
10 ExerciseTesting,165
Acknowledgments,xii StephenParidon
PartIII VentricularFunctioninSpecific
PartI Overview,1
Categories,185
1 PediatricCongenitalandAcquired
11 VentricularFunctioninPressureOverload
HeartDiseaseasItRelatesto
Lesions,187
VentricularFunctionand
StevenD.Colan
BloodFlow,3
MarkA.Fogel 12 VentricularFunctioninVolumeOverload
Lesions,205
2 VentricularFunction—TheBasicsofthe
StevenD.Colan
Discipline,11
MarkA.Fogel 13 TheRightVentricleinaDualChambered
Circulation,223
3 BloodFlow—TheBasicsof
AndrewN.Redington
theDiscipline,38
HiroumiKitajima&AjitP.Yoganathan 14 TheLeftVentricleinaDualChambered
4 HistoryandPhysicalExamination,55 Circulation,241
AmyH.Schultz&MarkA.Fogel DebraA.Dodd,AnnKavanaugh-McHugh,&
ThomasP.Graham,Jr.
5 PlainRadiographicExaminationoftheHeart
andChest,77 15 BloodFlowintheNormalandDiseased
JulienI.E.Hoffman&RobertC.Brasch Aorta,259
TheresaAnnTacy
6 CardiacCatheterization,84
JulienI.E.Hoffman 16 BloodFlowinNormalandDiseased
PulmonaryArteries,275
BrianD.Hanna
Part II Techniques in Assessing Ventricular
Function,99 17 ConsiderationsintheSingleVentricle,286
MarkA.Fogel
7 EchocardiographyandDoppler
Ultrasound,101 18 ExercisePerformanceinFontanPatients,309
JackRychik StephenParidon
8 MagneticResonanceImaging,124 19 ConsiderationsinIntensiveCare,314
MarkA.Fogel MerylS.Cohen
v
vi Contents
PartIV VentricularFunctionandBloodFlow 21 PharmacologyandVentricular
inSpecificCategories,325 Performance,345
StanfordEwing
20ConsiderationsinCatheterInterventions,327
MichaelCheung&LeeBenson Index,367
List of Contributors
LeeBenson,MD,FRCP(C),FSCAI ThomasP.GrahamJr,MD
TheDepartmentofPediatrics,DivisionofCardiology,The DirectorofPediatricCardiology,Ann&MonroeCarell
HospitalforSickChildren,TheUniversityofTorontoSchoolof ProfessorofPediatrics,TN37232,USA
Medicine,Toronto,Canada
BrianD.Hanna,MD,PhD
RobertC.Brasch
ClinicalAssociateProfessorofPediatrics,Universityof
ProfessorofRadiologyandPediatrics,UniversityofCalifornia, PennsylvaniaSchoolofMedicine,TheChildren’sHospitalof
California94143,USA Philadelphia,Philadelphia,PA19104,USA
MartinCharron,MD AmyH.Schultz,MD
DirectorNuclearMedicine,Children’sHospitalof
Instructor,DepartmentofPediatrics,Universityof
Philadelphia,andAssociateProfessorofRadiology,University
PennsylvaniaSchoolofMedicine,DivisionofCardiology,
ofPennsylvania,PA,19104-4399,USA
Children’sHospitalofPhiladelphia,Philadelphia,PA,USA
MichaelCheung,MB,ChB,MRCP(UK) JulienI.E.Hoffman,MD,FRCP(London)
TheDepartmentofPediatrics,DivisionofCardiology,The
ProfessorofPediatrics(Emeritus),DepartmentofPediatrics,
HospitalforSickChildren,TheUniversityofTorontoSchoolof
SeniorMemberoftheCardiovascularResearchInstitute,
Medicine,Toronto,Canada
UniversityofCalifornia,SanFrancisco,California94143,
USA
MerylS.Cohen,MD
AssistantProfessorofPediatrics,Director,Non-Invasive AnnKavanaugh-McHugh,MD
CardiovascularLaboratory,UniversityofPennsylvaniaSchool
AssistantProfessorofPediatrics,VanderbiltUniversity
ofMedicine,TheChildren’sHospitalofPhiladelphia,
MedicalCenter,TN37232,USA
Philadelphia,Pennsylvania,USA
StevenColan,MD HiroumiKitajima,MS
ProfessorofPediatrics,HarvardMedicalSchool,Director, WallaceH.CoulterDepartmentofBiomedicalEngine,Georgia
DivisionofNoninvasiveCadiology,Boston,MA,USA InstituteofTechnologyandEmoryUniversitySchoolof
Medicine,Atlanta,Georgia,USA
DebraDodd,MD
BruceY.Lee,MD
AssociateProfessorofPediatrics,DivisionofPediatric
DivisionofGeneralInternalMedicine,Universityof
Cardiology,VanderbiltUniversityMedicalCenter,Nashville,
Pennsylvania,1125BlockleyHall,423GuardionStreet,
TN37232,USA
Philadelphia,PA19104,USA
StanfordEwing,MDFAAPFRCP(C) StephenParidon,MD
ClinicalAssistantProfessor,UniversityofPennsylvania, AssociateProfessorofPediatrics,MedicalDirector,Exercise
DivisionofCardiology,34thSt.andCivicCenterBlvd, PhysiologyLaboratory,TheChildren’sHospitalof
Philadelphia,PA19104,USA Philadelphia,TheUniversityofPennsylvaniaSchoolof
Medicine,34thSt.andCivicCenterBlvd,Philadelphia,PA
MarkA.Fogel,MD,FACC,FAAP 19104,USA
AssociateProfessorofPediatricsandRadiology,Directorof
CardiacMRI,TheChildren’sHospitalofPhiladelphia,The AndrewRedington,MD,FRCP(C)(UK)
UniversityofPennsylvaniaSchoolofMedicine,34thSt.and Head,DivisionofCardiology,UniversityAvenue,Toronto,
CivicCenterBlvd,Philadelphia,PA19104,USA Ontario,M5G1X8,Canada
vii
viii ListofContributors
JackRychik,MD AjitP.Yoganathan,PhD
AssociateProfessorPediatrics,TheChildren’sHospitalof Regents’Professor,WallaceH.CoulterDepartmentof
Philadelphia,Philadelphia,PA19104,USA BiomedicalEngine,GeorgiaInstituteofTechnologyand
EmoryUniversitySchoolofMedicine,Atlanta,GA30332-0,
TheresaAnnTacy,MD USA
UniversityofCalifornia,SanFrancisco,USA
Foreword
ThoughflatteredbyDrFogel’sinvitation,itisawk- fluid dynamics could potentially open the field of
wardforasurgeontoprefaceabookwrittenbysuch virtualreality,oftailor-madesurgicalandinterven-
aneminentpanelofbioengineers,physiologists,car- tionalprocedures.
diologists,andclinicalinvestigators. Theunderstandingandpreventionoflateattri-
Havingacceptedthetask,however,Ihavefound tionoftheFontancirculationremainsanoutstand-
it very refreshing and most enjoyable to glance ingresearchquestionforthecardiovascularphysi-
throughthevariouschaptersofthisbookofapplied ologist.Acomprehensiveapproachtotheproblem
physiologytocongenitalheartdiseases.Itreminds must,however,includeathoroughinvestigationof
meoftheintellectualpleasureIexperiencedasaju- thesystemicvenousandthelymphaticcirculations,
niortraineewhenIreadAbrahamRudolph’sbook whichhavenotbeengiventheattentiontheydeserve
oncongenitaldiseasesoftheheart. intheliterature.
Majoradvancesininvasiveandnoninvasivemeth- Surgicalsuccessesofthepastfiftyyearshavecre-
ods of investigation of the cardiovascular system atedanewpopulationofpatients:adultswithop-
havecontributedgreatlytotheunderstandingofthe erated congenital heart disease. They now repre-
physiopathologyoftreatedanduntreatedcongeni- sentanewspectrumofresearchquestionsforthe
talmalformationsofthecardiovascularsystem.The cardiovascularphysiologist.Longitudinalfollow-up
impactoftheseinnovationsonthemanagementof studiesusingnoninvasivemethodsofinvestigation
congenitalheartdiseasesishighlightedthroughout are mandatory in order to provide these patients
thisbook.Itispleasingtonotethatachapter(Chap- with evidence-based treatment. There is today no
ter3)hasbeendedicatedtohistoryandphysicalex- scientificconsensuswithregardtothemanagement
amination,which,inanageofhightechnologyand of lesions as common as residual/recurrrent mild
multidisciplinaryteamapproach,aretoooftenfor- tomoderatecoarctationoftheaortaorpulmonary
gottenorunderestimated.Itisareminderthatour valveregurgitationlateafterrepairoftetralogyof
firstdutyistoimprovequalityoflifeandlongevity. Fallot.
Theemphasisonexerciseperformance(Chapters10 Becauseofmylifelonginterestincardiovascular
and18)ismostappropriateinthatcontext. physiologyIamprobablybiasedinmyacclamation
Abookonthephysiologicalapproachtotheman- ofthisbook.Itismybeliefthatphysiologyis,andwill
agementofcongenitalheartdefectsisrationaland remain,thehubofallsciencesdealingwithcongen-
timely.Thefollowingarejustafewexamples. ital heart defects. Clinical results will continue to
Abetterunderstandingofrightventricularphys- beassessedbyphysiologicalmeasurements.Future
iology has helped to elucidate the arcanes of low advancesinpharmacology,electrophysiology,inter-
cardiacoutputsyndromeafterrepairoftetralogyof ventionalandsurgicalprocedures,aswellasinbasic
Fallot. A more accurate assessment of ventricular sciencessuchascellularandmolecularbiology,gene
functioninvolumeorpressureoverloadsituations therapy,etc,willinevitablybetranslatedintophysi-
hascontributedtotherefinementofindicationsand ologicalimprovements.
timingforintervention. DrFogelandhiscoauthorsmustbecongratulated
Theevolutionofstrategiestopalliateuniventricu- fortheirtourdeforce.Thisbookwillattractawide
larhearts,fromtheNorwoodoperationtothevariety audience,fromtraineestopractitionersandscien-
ofFontanprocedures,hasbeenanexhibitionofap- tists.
pliedphysiologytoclinicalpractice.Mathematical MarcRdeLevalMDFRCS
modelinghasbeenhelpfulinoptimizingtheflowdy- ProfessorofCardiothoracicSurgery
namicsofsomeoftheseprocedures.Themarriage GreatOrmondStreetHospitalfor
ofmagneticresonanceimagingandcomputational ChildrenNHSTrust,London,UK
ix
Preface
Congenitalheartdiseaseisoneofthemajorcauses gleanedfrommultiplesources,whicharenotto-
ofmorbidityandmortalityinpediatrics.Inaddition, tallydevotedtothistopic(e.g.,ashortchapterina
becauseofmajoradvancesoverthepast50years, textbook).Assuch,allthenuancesarenottotally
moreandmoreofthesechildrenaregrowinginto explained.
adulthoodandrequiremedicalattentionandfollow- Itwouldhavebeeninstructiveandmuchmoreeffi-
up. It is therefore incumbent, not only on the pe- cient,Ithought,tohavehadthoroughsummaries
diatrician, pediatric cardiologist, cardiac surgeon, ofalltheaspectsofventricularfunctionandblood
orcardiacanesthesiologisttofamiliarizethemselves flowinoneplacethatisdevotedsolelytocongenital
withallthenuancesofthisbranchofpediatrics,but heartdisease.Theideaforthistextbookistoallow
thefamilypractitioner,internist,adultcardiologist, thetrainee,practitioner,andtheresearchertohavea
andintensivistalsoneedtohaveaworkingknowl- repositoryofinformationtobasetheirfundamental
edgeofthisaswell. understanding,practice,andexperimentson.
Tounderstandcongenitalheartdiseaseistoalso Anytextonthissubjectmustbeorganizedina
understandthatitisoneofthemoretechnicaldis- logicalwayforthereadertogetacompleteunder-
ciplineswithinpediatricsandmedicineingeneral. standingoftheissuesinvolved.Towardsthatend,
This is based on the continued evolution of diag- the textbook is divided into three major sections.
nosis and management grounded on advances in Thefirstinvolvesfamiliarizingthereaderwiththe
technology. With the advent of advanced imaging minimum tools needed to understand ventricular
techniques, invasive monitoring, and many other functionandbloodflowinthisbookandelsewhere,
breakthroughs,ourknowledgeofthisfieldisvastly andhence,theOverviewsectionwithitsBasicsof
differentnowthanitwasjust10yearsago.Butcon- theDisciplinechapters.Thissectionalsoincludesa
genitalheartdiseaseisnotjusttechnicalinthatre- lengthy“justification”fortheneedforsuchatext-
spect; complex anatomy, physiology, hemodynam- bookasthoseunfamiliarwiththedisciplinemayre-
ics,ventricularfunction,andbloodflowalongwith quire.Thenextsectiondiscussesthevarioustech-
theirinteractionswitheachotherformanintricate niquesandtechnologyusedinevaluatinghowwell
webofconnectionsandassociationsthatneedtobe theheartpumpsandthewaybloodflows.Specifi-
deciphered.Basicprinciplesneedtobeappliedsothat cally,thesechaptersfocusonthesetechniquesand
anyoneofthevariedtypesofcongenitalheartdis- technologiesastheyrelatetopediatrics.Finally,the
easethatpresenttothepractitionercanbeanalyzed thirdandlargestsectiondealswithbroadcategories
andareasonableplanofactioncanbeimplemented. ofcongenitalheartdiseaseonthissubject.Although
Inaddition,precisedefinitionsmustalwaysbeused there are many ways to organize and categorize
to assure correct communication of what is truly congenital heart disease, I thought it best to sort
goingoninthepatient. itboth“physiologically”(i.e.,PressureOverloadLe-
Withincongenitalheartdisease,ventricularfunc- sions,VolumeOverloadLesions)aswellas“anatomi-
tionandbloodflowisadisciplinethatisveryexacting cally”(e.g.,TheRightVentricleinaDualChambered
andyetsomisunderstoodandmisused.Theremay Circulation).Therearealsoadditionalchapterson
betwofundamentalreasonsforthis: special topics such as the intensive care unit and
(cid:2)
Asindividualswhoworkinthisfield,weborrowa pharmacology.
lotfromouradultcolleagueswhenitsapplicability Becauseofspaceandtimeconstraints,thistext-
maynotbewhollyappropriate. bookcannotbeencyclopedic.Indeed,issuesrelated
(cid:2)
Whatwelearnaspediatricresidents,cardiology tomolecularbiology,genetics,etchavenotbeenex-
fellows, and as an attending on this subject is plored.Ihaveendeavoredtomaketheformatofthis
x