Citation for published version (APA): Luijendijk, P. (2014). Aortic coarctation: late complications and treatment strategies.

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1 UvA-DARE (Digital Academic Repository) Aortic coarctation: late complications and treatment strategies Luijendijk, P. Link to publication Citation for published version (APA): Luijendijk, P. (2014). Aortic coarctation: late complications and treatment strategies. General rights It is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an open content license (like Creative Commons). Disclaimer/Complaints regulations If you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible. UvA-DARE is a service provided by the library of the University of Amsterdam ( Download date: 27 Mar 2019

2 Case report

3 Abstract Aorticcoarctationisacommoncongenitalcardiacdefect,whichcanbediagnosedoverawiderange ofagesandwithvaryingdegreesofseverity.wepresenttwocasesofpatientsdiagnosedwithaortic coarctation in adulthood. Both patients were treated by an endovascular approach. These cases demonstratethevarietyofindicationsinwhichpercutaneoustreatmentisanexcellentalternative forsurgicaltreatmentinadultnativecoarctationpatients.

4 Introduction Aorticcoarctationisacongenitalcardiovascularmalformationcomprisingacircumscriptnarrowing oftheaorticlumenusuallylocateddistallyoftheleftsubclavianartery,atthesitewheretheductus arteriosusenterstheaorta.aorticcoarctationaccountsfor58%ofallcongenitalheartdefectsand may be associated with other leftsided cardiac abnormalities, such as bicuspid aortic valve, hypoplastic aortic arch, and ventricular septal defect. When diagnosed in neonates, surgical reconstruction is the treatment of choice. In infants, surgery is preferred as well, but balloon angioplastyhasalsoevolvedrapidlyoverthepastdecade.bothtreatmentoptionscarrytheriskof restenosis and late aneurysm formation. 1;2 For recoarctation after initial surgical treatment, good longtermresultshavebeenreported. 3;4 Asmallproportionofaorticcoarctationisnotdiagnoseduntiladolescenceoradultage.The presenting symptoms usually include hypertension, congestive heart failure, or intermittent claudication.thereisanindicationfortreatmentifthegradientis>20mmhgincombinationwith hypertension,eitheratrestorduringexercise. 5 Wheninterventioniswarranted,theriskofsurgeryis considerablebecauseoftheextensivecollateralnetworksurroundingthecoarctationwhichresults in a high risk of bleeding complications. As a consequence, balloon angioplasty with or without stentinghasevolvedasatreatmentmodalityforadolescentswithnativeaorticcoarctation. 6;7 CaseA.Nativeaorticcoarctation Thefirstcasedescribesamalepatientreferredtoourhospital,whohadbeendiagnosedwithsevere aortic coarctation at the age of 24 in the Middle East. At the time of diagnosis, the gradient measuredbycatheterizationwas90mmhg.thecoarctationwasleftuntreateddespitethepresence ofhypertension.hepresentedtoourhospitalattheageof33withfatigue,shortnessofbreathon exercise,complaintsofheadache,andintermittentchestpainbothatrestandduringexercise.his bloodpressurewas160/100mmhg.onauscultation,normalheartsoundswereheard,aswellasan ejectionmurmurgradeii/viattherightsecondintercostalspaceandalatesystolicmurmurgrade III/VIattheapex.Palpationofthefemoralarteriesrevealedslightlydecreasedpulsations.TheECG showed a left anterior hemiblock. Echocardiography showed mild concentric left ventricular hypertrophywithoutdilatation.atricuspidaorticvalvewaspresentwithmildaorticregurgitation. Theaorticrootwasdilatedwithadiameterof41mm.Thediameteroftheascendingaortawas32 mm. Cardiac magnetic resonance imaging (MRI) confirmed a complete interruption of the descendingaortadirectlyafterthedilatedleftsubclavianartery.distalfromtheocclusion,theaorta was supported by many collaterals originating from the left mammary artery and the intercostal arteries. (Figure 1a and 1b) It was decided to treat this patient with a percutaneous balloon expandable 28 mm covered stent. Retrograde passage proved impossible, but using the radial approach balloon passage successful. After stent implantation with deployment to 18 mm, angiographyrevealedawellexpandedstentandtherewasnoresidualpressuregradient(figure2a and 2b) (Tape a and b). Two months later, the patient was without complaints and followup angiographyshowedgoodstentposition,noaorticaneurysmsandtherewasnopressuregradient. 4

5 ŽƌƟĐĐŽĂƌĐƚĂƟŽŶ >ĂƚĞĐŽŵƉůŝĐĂƟŽŶƐĂŶĚƚƌĞĂƚŵĞŶƚƐƚƌĂƚĞŐŝĞƐ a. b. Figure1:Magneticresonanceimaginginnativeaorticcoarctation a.mrireconstructionimagedemonstratingthemanycollateralarteries b.lategadoliniummriimagedemonstratingtheseverecoarctationaccompaniedbythecollateralarteries a. b. Figure2:PercutaneousstentͲimplantationforaorticcoarctation a.angiographicimagebeforestentexpansionincasea b.angiographicimageafterstentexpansionincasea ϲϲ

6 CaseB.Nativeaorticcoarctation Thesecondcasedescribesa43yearoldmanwhohadrecentlybeendiagnosedwithsevereaortic coarctation and a hypoplastic aortic arch, accompanied by a bicuspid aortic valve and moderate aorticvalveregurgitation.inaddition,hehadadilatedleftventriclewithimpairedsystolicfunction. Headmittedseverealcoholandcannabisabuseoverthepastseveralyears. He presented with increasing fatigue but no exertional dyspnea or orthopnea. Physical examination revealed an important blood pressure difference between right and left arm (125/77 versus 99/72 mmhg, respectively). On auscultation normal heart sounds with a grade II/IV crescendo/decrescendo murmur and a short diastolic murmur were heard. Palpation revealed a palpable liver 3 cm below the diaphragm, and weak peripheral pulsations. The electrocardiogram showedmildintraventricularconductiondelay(qrs120ms),andleftventricularhypertrophywith secondary repolarisation abnormalities. Echocardiography showed a dilated, hypertrophied left ventricle with a moderately impaired function (Figure 3a). Diffuse left ventricular wall motion disturbanceswithhypoandakinesisoftheinterventricularseptumwereseen.theaorticvalvewas bicuspid with a maximal gradient of 26 mmhg and mild aortic regurgitation. The aortic root and ascending aortic diameters were 39 mm and 33 mm respectively. Turbulent flow was seen in the descendingaortawithamaximumvelocityof3.5m/sandatypicalsawtoothshape(figure3b).this patientwastreatedwithballoondilatationandimplantationofa22mmcoveredstent.beforethe procedure,meansystolicbloodpressureswere70mmhgproximaltothecoarctationand45mmhg distalofthecoarctation.stentimplantationwassuccessfulandresultedinpressureequilibration. 4 a. b. Figure3:TransthoracicechocardiographyincaseB a.apicalfourchamberviewdemonstratingthedilatedleftventricle b.thetypicalsawtoothpatternwithcontinuousflowoncontinuouswavedopplerinterrogationinaortic coarctation

7 Discussion Coarctation of the aorta can be diagnosed across a wide age spectrum and with a range of symptoms.anuntreated coarctationoftheaortapresentinglaterinlifehasanegativeimpacton longtermsurvival,ascomparedtopatientsdiagnosedandtreatedinearlylife. 8 Latecomplications such as aortic aneurysm formation, infective endocarditis, hypertension, premature (coronary) atherosclerosis and cerebrovascular accidents all account for the increased morbidity and mortality. 9;10 The two patients described above were both diagnosed in adulthood. Traditionally, surgicalrepairhasbeenaneffectivetreatmentoptionforadultpatientswithnativecoarctation,and it has been shown to improve the efficacy of postoperative antihypertensive treatment. Adult patients with native coarctation are usually at lower operative risk than recoarctation patients becausetherearenoconcernsrelatedtopleuralorperiaorticscartissueassociatedwithprevious surgical repair. However, these patients do have many associated cardiovascular comorbidities includingleftventricularhypertrophyandarrhythmias. 8 Thefirstcasepresentedwithasevereaortic coarctation,withmanycollateralsduetothesevereobstruction.inthesecondcasethesevereaortic coarctationwasaccompaniedbyahypoplasticarchandanimpairedleftventricularfunctionpossibly duetolonglastingalcoholabuse.inbothcases,theriskofcardiacsurgerywasestimatedtobetoo high.incase1,theextensivecollateralnetworksurroundingthecoarctationcausedasubstantialrisk of perioperative bleeding complications. In case 2 surgical risk was increased because of the impaired left ventricular function. In both cases endovascular repair with balloon angioplasty and stent placement was performed. Endovascular repair with or without stenting has been demonstrated to be an acceptable alternative to surgical repair with similar outcome in native coarctation. The use of covered stents has been promoted to avoid vascular complications. 8 The incidenceofrecoarctationafterstentplacementislowerthanafterballoonangioplastyalone,dueto alowerdegreeofelasticrecoilandavoidanceofvesseloverdilationand,asaconsequencereduced aorticinjury.concernsthatariseincoarctationpatientsafterendovascularrepairarestrutfractures, metalfatigueoraorticdeteriorationoraorticdisruptionatthecoarctationsite. 11 However,studies documenting the longterm outcomes of stentimplantation confirmed the low rate of procedure related adverse events and a longterm procedural success in both native and recurrent aortic coarctationoraneurysmaldisease. 12;13 Longtermoutcomewascomparablewithothersurgicaland interventional modalities. This case report demonstrates the variety of indications in which percutaneous treatment is preferable to surgical treatment in adult patients with native aortic coarctation.moreresearchonthelongtermassessmentofadultnativecoarctationpatientsafter percutaneousinterventionwillbeimportanttodeterminetheimpactonsurvivalinthesepatients.

8 ReferenceList (1) McElhinneyDB,YangSG,HogartyANetal.Recurrentarchobstructionafterrepairofisolatedcoarctationofthe aortainneonatesandyounginfants:islowweightariskfactor?jthoraccardiovascsurg2001;122: (2) RaoPS,GalalO,SmithPA,WilsonAD.Fivetonineyearfollowupresultsofballoonangioplastyofnativeaortic coarctationininfantsandchildren.jamcollcardiol1996;27: (3) Ince H, Petzsch M, Rehders T et al. Percutaneous endovascular repair of aneurysm after previous coarctation surgery.circulation2003;108: (4) Reich O, Tax P, Bartakova H et al. Longterm (up to 20 years) results of percutaneous balloon angioplasty of recurrentaorticcoarctationwithoutuseofstents.eurheartj2008;29: (5) BaumgartnerH,BonhoefferP,DeGrootNMetal.ESCGuidelinesforthemanagementofgrownupcongenital heartdisease(newversion2010).eurheartj2010;31: (6) PaddonAJ,NicholsonAA,EttlesDF,TravisSJ,DyetJF.LongtermfollowUpofpercutaneousballoonangioplastyin adultaorticcoarctation.cardiovascinterventradiol2000;23: (7) PedraCA,FontesVF,EstevesCAetal.Stentingvs.balloonangioplastyfordiscreteunoperatedcoarctationofthe aortainadolescentsandadults.cathetercardiovascinterv2005;64: (8) WheatleyGH,III,KoulliasGJ,RodriguezLopezJA,RamaiahVG,DiethrichEB.Isendovascularrepairthenewgold standardforprimaryadultcoarctation?eurjcardiothoracsurg2010;38: (9) Vriend JW, Mulder BJ. Late complications in patients after repair of aortic coarctation: implications for management.intjcardiol2005;101: (10) VriendJW,deGrootE,deWaalTT,ZijtaFM,KasteleinJJ,MulderBJ.Increasedcarotidandfemoralintimamedia thicknessinpatientsafterrepairofaorticcoarctation:influenceofearlyrepair.amheartj2006;151: (11) TanousD,CollinsN,DehghaniP,BensonLN,HorlickEM.Coveredstentsinthemanagementofcoarctationofthe aortaintheadult:initialresultsand1yearangiographicandhemodynamicfollowup.intjcardiol2010;140: (12) HolzerR,QureshiS,GhasemiAetal.Stentingofaorticcoarctation:acute,intermediate,andlongtermresultsof a prospective multiinstitutional registrycongenital Cardiovascular Interventional Study Consortium (CCISC). CatheterCardiovascInterv2010;76: (13) Shennib H, RodriguezLopez J, Ramaiah V et al. Endovascular management of adult coarctation and its complications:intermediateresultsinacohortof22patients.eurjcardiothoracsurg2010;37:

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