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C H A P T E R 7
PulmonaryArteryStenosis
M.AbigailSimmonsMDandJeremyD.AsnesMD
I.Introduction
II.EtiologyofPulmonaryArteryStenosis
III.Pathophysiology
IV.InterventionalStrategies
A.FirstBalloonAngioplastyandAdvancements
B.IndicationsforIntervention
V.GoalsofTherapyandMeasuresofSuccess
VI.StandardBalloonAngioplasty
A.BalloonExpansion
B.TypesofBalloonAngioplastyCatheters
VII.CuttingBalloonAngioplasty
A.Complications
B.ProceduralConsiderations
VIII.StentAngioplasty
B.AdditionalConsiderations
IX.StentSelection
A.CommonlyUsedStents
D.TechniqueConsiderations
E.Complications
X.TherapeuticAlgorithm
KeyPoints
■Pulmonaryarterystenosismaybeduetocongenitaloracquireddisease.
■Pulmonaryarterystenosisshouldbeconsideredaspartofthedifferentialdiagnosisforany
adultundergoingevaluationofpulmonaryhypertension.
■Indicationstointerveneon pulmonaryarterystenosisinclude:1)rightventricularsystolic
pressure greater than 2/3 systemic pressure, 2) Significant stenosis in the setting of right
ventriculardysfunction,3) pulmonaryarterystenosiswithamarkedperfusioninequalityor
deficit, 4) regional pulmonary artery hypertension in unaffected lung segment, and 5)
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pulmonaryarterynarrowing/distortioninpatientswithcavopulmonaryanastomoses.
■Interventional techniques to address pulmonary artery stenosis include simple balloon
angioplasty,cuttingballoonangioplasty,andstentangioplasty.
I.Introduction
A.Obstructionsorstenoseswiththepulmonaryarterialtreeresultfromadiversegroupofintrinsicand
extrinsicfactors.
B.Pulmonaryarterystenosesmayinvolvethemainpulmonaryartery,theleftandrightbranchpulmonary
arteries,andthelobar,segmental,andsubsegmentalbranchesofthemoredistalpulmonaryarterialtree.
Obstruction may be isolated to a single vessel or may occur at multiple sites and multiple levels.
1
Management strategies for pulmonary artery stenosis include balloon angioplasty, cutting balloon
angioplasty, and stent angioplasty. Patient-specificstrategiesdependonetiology,lesioncharacteristics,
andpatientcharacteristics.
II.EtiologyofPulmonaryArteryStenosis
A.Pulmonaryarterystenosiscanbebroadlyclassifiedaseithercongenitaloracquired(Table7.1).Ina
studybyFranch, 60% ofcongenital stenoses were found inconjunction withcongenital heart disease
while 40% were isolated. Isolated congenital stenosis is often found in association with genetic
syndromesincludingWilliams,Alagille,andNoonansyndrome(Fig.7.1).Inthesesettings,thestenoses
tendtobediffuse,involvingmultiplesegmentsatmultiplelevelsofthepulmonarytree.
Table7.1
EtiologiesofPulmonaryArteryStenosis
Conge nitalMalformationofPulmonaryArterialSystem
■Congenitalheartdiseaseassociated
■TetralogyofFallot,pulmonaryatresia,pulmonaryvalvarstenosis
■Main,branch,orlobararterystenoses
■Isolatedpulmonaryarterystenosis
■Mainorbranchpulmonaryarterystenosis
GeneticSyndromes
■Williamssyndrome:
■Diffuseinvolvement:branch,lobar,segmentalpulmonaryarteries
■Alagillesyndrome:Peripheralpulmonaryarterystenosis
Postsurgical
■Distaltositeofpulmonaryarterypatchoranastomosis
■SiteofpreviousBlalock-Taussigshunt
■Siteofpatentductusarteriosusligationordeviceimplantation
■Followingremovalofpulmonaryarteryband
■Followingarterialswitchoperation
IdiopathicPulmonaryArteryStenos is
■Takayasuarteritis:Main,branch,andlobarpulmonaryarterystenosis
■Fibrosingmediastinitis:Mainandbranchpulmonaryarterystenosis
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Exte rnalCompress ion
■Compressionfromtumor(iebronchogeniccarcinoma)orlymphadenopathy
■Compressionfrominfiltrativeorfibroticlungdisease(iesarcoidosis)
FIGURE7.1 AandB,Anterior-posteriorandlateralangiogramsfromapatientwithWilliamssyndrome.Thereis
diffusediseasewithhypoplasiaofthecentralpulmonaryarteriesandseverestenosesofalmostalllobarand
segmentalbranches.C,Severediscreterightpulmonaryarterystenosis(whitearrow)inapatientwithTakayasu
arteritis.D,Severeproximalpulmonaryarterystenosis/hypoplasiainapatientwithAlagillesyndrome.
B.Pulmonaryarterystenosesmaybeacquiredasaresultofcongenitalheartdiseasesurgery,particularly
ifthe surgery involvesmanipulation ofthe pulmonary arteries themselves. Patchmaterial, suture lines,
anddistortion due to kinking or stretchingof the pulmonary vessel may all contribute to postsurgical
pulmonaryarterystenosis(Figs.7.2A–Cand7.3A).
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FIGURE7.2 AandB,Stenosisduetofoldingoftheproximalleftpulmonaryarteryatthesiteofasurgicalpatch
before(A)andafter(B)stentplacement.CandD,Severerightpulmonaryarterystenosisrelatedtosurgicalpatch
materialbefore(C)andafter(D)stentplacementandtranscathetervalve-in-valvepulmonaryvalveimplant.D,
Pulmonaryarteryangiograminapatientwithtranspositionofthegreatarteriesfollowingarterialswitchoperation.
Therearestenosesofthelobarbranchesrelatedtosurgicalmanipulationofthepulmonaryvasculature(E)
successfullytreatedwithstentangioplasty(F).
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FIGURE7.3 Balloonangioplastyofproximalleftpulmonaryarterystenosisrelatedtosurgicalpatchaugmentation,
before(A)andfollowing(D)successfulballoonangioplastyinapatientwithtetralogyofFallot.Notethediscrete
waistintheangioplastyballoon(B)thatresolveswithhigh-pressureinflation(C).
C.Pulmonaryarterystenosis presentingdenovointhe adult is arare but important condition and is
usually an acquired pathology.
2,3
Etiologies include external compression from tumor or
lymphadenopathy,fibrosingmediastinitis,systemic vasculitis (egTakayasuarteritis orBehcetdisease),
thromboembolic disease, and sarcoidosis. (Table 7.1). These are often misdiagnosed as idiopathic
pulmonary arterial hypertension or pulmonaryhypertensionduetochronicvenousthromboembolism.In
cases oflatediagnosis, patients haveoften receivedinappropriateor incomplete therapeutic strategies
withlittleclinicalbenefit.
III.Pathophysiology
Pulmonary artery stenosis results in varying degrees of ventilation/perfusion mismatch, pulmonary
hypertension,vascularinjuryinunaffectedsegments,elevationsofrightheartpressure,rightventricular
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dysfunction,andlimitationsofcardiacoutput.Inthepresenceofanintra-cardiacshunt(eg,patentforamen
ovale,atrialseptaldefect,orventricularseptaldefect)obstructiontopulmonarybloodflowcanresultin
cyanosisfromshuntingofdeoxygenatedbloodtothesystemiccirculationduetoincreasedresistanceto
pulmonary blood flow. Untreated, pulmonary artery stenosis can contribute to exercise limitation,
diminished quality oflife,and,in thecase ofcongenital lesions and stenosis due toprevious cardiac
surgery,limitedpulmonaryvasculargrowthandpost-operativemortality.
4,5
IV.InterventionalStrategies
A.FirstBalloonAngioplastyandAdvancements
Locketal,werethefirsttoperformballoonangioplastyofpulmonaryarteries.Theirseminalworkwith
anexperimentallambmodelwasquicklytranslatedtothecongenitalcardiaccatheterizationlaboratory.
6,7
Rapid advancements incatheter, balloon, stent and guidewire technologies, as well as refinements in
angioplasty techniques, broadened the scope of lesions that can be safely treated with transcatheter
therapies.Inthecurrentera,simpleballoonangioplasty,cuttingballoonangioplasty,andstentangioplasty
aretheprimaryinterventionsforpulmonaryarterialstenosis.
B.IndicationsforIntervention
Generallyagreeduponindicationsforinterventionare
8,9
1.Significantelevationinrightventricularsystolicpressures(≥2/3systemic)
2.Significantstenosisinthesettingofrightventriculardysfunction
3.Pulmonaryarterystenosiswithamarkedperfusioninequalityordeficit
4. Regional pulmonary artery hypertension in unaffected lung segments (mean distal pressure
>25mmHg)
5.Pulmonaryarterynarrowing/distortioninpatientswithcavopulmonaryanastomoses
V.GoalsofTherapyandMeasuresofSuccess
The goals of intervention include reduction in right ventricle pressure, improved distribution of
pulmonary blood flow, preservation and improved growth of distal pulmonary vasculature, pressure
reduction in unobstructed lung segments,andimprovementinexercise capacity. Therapeuticsuccessis
measuredbyangiographicand/orclinical/physiologicimprovement.10Nuclearperfusionimaging helps
assessregionalpulmonaryperfusionandtheneedforandsuccessofanintervention.
VI.StandardBalloonAngioplasty
Successfulballoonangioplastytearstheintimaandmediaofthepulmonaryarterialwall.
11,12
A.BalloonExpansion
1.Ingeneral,balloonseitherexpanduniformlywithadiscretewaistatthesiteofhighestresistance,or
nonuniformlywithoutadiscretewaist.
2.Thelatterisoftenassociatedwithstenosisduetoexternalcompression,kinking,orstretchingofthe
vessel.
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3.
Thesevesselstypicallyexhibitsignificantrecoillimitingtherapeuticbenefitofballoonangiogplasty.
4.
Whenawaistispresent,successfulangioplastyisdependentonitseradication.
13
5.
Angiographyfollowingsuccessfulballoonangioplastyoftenshowsanon-obstructiveintra-luminalfilling
defectindicativeofanappropriateintimalandmedialtear.
B.TypesofBalloonAngioplastyCatheters
A wide array of balloon angioplasty catheters is available for pulmonary artery balloon angioplasty.
Balloonselectiondependsonbothpatientandtargetvesselcharacteristicswithdifferencesinguidewire
size, shaft flexibility, balloon compliance, balloon and balloon shoulder length, and nominal/maximal
inflationpressureimpactingangioplastycatheterchoice.
1.Low-pressureballoonangioplasty.Low-pressureballoonangioplasty(4-10atm)usingballoons2-4
timeslargerthanthetargetminimallumendiameterhasbeensuccessfulinupto60%oflesions.
13,14
2.High-pressureballoonangioplasty.Successrateswithhigh-pressureballoonangioplasty(10-22atm)
exceed those seen with low-pressure balloons. Furthermore, successful high-pressure angioplasty is
frequently achieved at lower balloon:minimal lumen ratios than those needed for low-pressure
angioplasty.15Thus,withhigh-pressureangioplasty,amoreconservativeapproach,startingwithratiosof
2-3:1,withincrementalincreasesintheabsenceofsuccessmaybepreferable.
3.Inrecentstudies,uptoone-thirdofpulmonaryarterystenosesremainresistanttohigh-pressureballoon
angioplasty.9 Resistance to angioplastyis more common in distal pulmonaryarteries while proximal
vesselsexhibithigherratesofrecoil.Thisislikelyrelatedtothevariablemechanismsofstenosisatthese
sites.Restenosisratesof10%-35%havebeenreportedfollowingsimpleballoonangioplasty.
9,16
VII.CuttingBalloonAngioplasty
Cuttingballoonangioplastyforpulmonaryarterystenosiswasfirstreportedin199917andhassincebeen
reported in multiple series and studied in a randomizedtrial comparing cutting balloonangioplasty to
high-pressure balloon angioplasty.18 Cutting balloons improve the overall success rate for pulmonary
artery angioplasty, particularly for lesions resistant to standard high-pressure balloon dilation. The
microsurgicalbladesofthecuttingballooncreatepreciselongitudinal“incisions”alongthelengthofthe
targetlesion.Theincisionsareformedatlowerpressuresthanthoserequiredtocreateanintimal/medial
tearwithastandardangioplastyballoon,andthuscuttingballoonsmayreducetheriskofvesselrupture.
Resultsare bestin pulmonary arterystenosesthatexhibita discretewaistduring standardangioplasty.
Success in long-segment stenoses, diffusely hypoplastic pulmonary arteries, and pulmonary arteries
exhibitingsignificantrecoilislimited.
19
Small diameter, 6-, 10-, and 15-mm long cutting balloons are available from 2.0 mm to 4.0 mm in
diameterin0.25mmincrements(Flextome,BostonScientific).Theseareavailableasbothover-the-wire
andmonorail systems. Importantly, theseonly accepta 0.014″ guidewire; thus guidewire exchangeis
frequently required when switching from a standard angioplasty balloon to a small diameter cutting
balloon.Largediametercutting balloonsarelimitedto2 cmlengthsandareavailable from 5-8mmin
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diameterin1mmincrements.Theseballoonsrequirea0.018″guidewire.
Aswith standardballoonangioplasty,cuttingballoonsizeselectionis dependenton the minimallumen
diameterofthetargetlesionandthediameteroftheadjacentnormalvessel.Successfuldilationfrequently
requiresballoondiameters200%-400%oftheminimallumendiameter.However,theballoonshouldnot
be>1-2mmlargerthantheadjacentnormalvessel.Someoperatorsrecommendchoosingcuttingballoon
diametersbasedonthediameterofthestandardangioplastyballoonwaistat8-15atm,selectingaballoon
0.5-1.0mmlargerthanthewaist.10Cuttingballoondilationcanthenbefollowedbyangioplastywitha
slightlylargerstandardballoonthusreducingtheriskofinjurytoadjacentnormalvasculature.
A.Complications
Althoughballoonangioplastyisa relativelysafeprocedure,thereare severalcomplicationswhich the
interventionalist should be aware of and prepared to address. Reported complications of balloon
angioplastyinclude:death,pulmonaryedema,pneumothorax,hemoptysis,arrhythmia,hypotension,stroke,
accesssiteinjury,cardiacarrest,targetvesselaneurysmformation,occlusiveintimalflaps,containedand
uncontained transmural pulmonary artery tears.10 Death is most commonly due to uncontained tears.
Mortalityratesof1%to9% were reportedinearlycase series.However,refinementintechniqueand
improvementinavailabletechnologieshavereducedtheriskofdeath.
Severalfactorsincreasetheriskofvesseltears.Balloonsexceedingtwicethediameteroftheproximalor
distal “normal”vesselresult insignificantover-distension andincrease theriskforvessel ruptureand
aneurysmformation, particularlyin the smallerdistalvessel.Proximal movementoftheballoonduring
inflationmayindicateoversizing,andcontinuedforceddilationmayresultinvesselrupture.Riskisalso
increasedinthesettingofseverepulmonaryhypertension.
In the case of vessel rupture, coil embolization and covered stent implant may be life saving.
20
Techniquestoaddressanobstructingintimalflapinclude:coveredorbaremetalstentimplantation.
Reperfusion injury due to the sudden increase in flow and pressure that accompanies a successful
angioplastycanresultinregionalpulmonaryedemainthesegmentsubtendedbythedilatedvessel.This
canrequireprolongedmechanicalventilationanddiuretictherapybuttypicallyresolveswithin72hours.
10,21
Inthesettingofsignificantventilationperfusionmismatch,impairedgasexchangeduetoedemacan
besevereandevenlethal.Increasesindistalmeanpressureto>20mmHgor>150%changeinpressure
areassociatedwithdevelopmentofedema.
21
B.ProceduralConsiderations
1. General anesthesia allows for airway control/clearance in the setting of pulmonary edema or
hemorrhageandimprovedpatientcomfortandpositioningduringlongprocedures
2.Cross-matchedbloodshouldbeimmediatelyavailable
3. Guidewires should be positionedinthelargestdistal branchpossible to minimizedistention of the
distalvesselbytheangioplastyballoon
4.Balloondeploymentviaalongsheathpositionedjustproximaltotheangioplastysiteallowsfor:rapid
angiography between dilations; rapid assessment of pressure; rapid deployment of coils, occlusion
devices,andstentsifnecessary;shieldingofcuttingballoonmicrotomesduringpassagethroughtheheart;
andenhancedstabilityoftheballoonduringinflation
5.Stenoseslimitingflowtothelargestsegmentoflungshouldbeaddressedfirst.Ifmoredistalstenoses
arepresent,theseshouldbeaddressedfirsttolimitpassagethroughpreviouslydilatedsegmentsandto
allowfortimelyrecognitionofreperfusionedema
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