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Forfurtherinformationpleasecontact:
OmanSocietyofLipidandAtherosclerosis(OSLA)KhalidAl-RasadiPresidentE-mail:[email protected]
InternationalAtherosclerosisSociety(IAS)EmanuelaFolcoExecutiveDirectorE-mail:[email protected]
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Onbehalf of the InternationalAtherosclerosis Society (IAS), theOmanSocietyofLipidandAtherosclerosis(OSLA),theGulfHeartAssociation(GHA),andtheEmiratesCardiacSociety(ECS),wewouldliketowelcomeyoutoDubai,UAEandtheCourseonIdentifyingandTreatingSevereFamilialHypercholesterolemiaheldonOctober14-15,2017.
Thepurposeofthisclosed-numberresidentialCourseistoincreasetheknowledgeand experience of practicing clinicians from the Gulf Region interested in themanagement of lipid disorders. In particular the Course will discuss how torecognize, diagnose, and treat severe familial hypercholesterolemia (both HeFHandHoFH)inpatientsinaccordancewithcurrentguidelinesandwillconsistoftwodaysoflecturesandinteractivesessions.
This Course will offer a unique opportunity to learn from the prestigiousinternational,regional,andnationalfacultyaswellasyourpeers.WeencourageyoutoactivelyparticipatetogainthemostfromtheCourseandtousethetimetobuildcollegialrelationshipswiththefacultyandotherparticipants.
Thebenefitstobederivedfromyourinvolvementoverthenext3dayswillnotonlyenhanceyourcareerandyourinstitutionbutwillbeultimatelyallowyoutoprovidebettercareforyourpatients,thusimprovingthehealthcareoftheMENAregion.
Please feel free to reach out to any of the faculty members if you have anyquestions.
WearealllookingforwardtoaveryproductiveandsuccessfulCourse.
Bestregards,
RaulSantosPresident-Elect,IAS
KhalidAl-RasadiPresident,OSLA
WaelAlmahmeedBoardmember,GHA
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The INTERNATIONAL ATHEROSCLEROSIS SOCIETY (IAS) as a federation of 64nationalandregionalmemberorganizationsworldwide(representing54countries)ispoisedtofacethechallengesofthenewcenturypresentedbythemanyscientificadvances in the field, the ageing of the population in most countries, and thegrowingneedforeducationofthoseinthemedicalandhealthcareprofessionsandofthelaypopulation.
The IAS exists to coordinate the exchange of scientific information among theconstituentsocieties,tofosterresearchintothedevelopmentofatherosclerosis,andtohelptranslatethisknowledgeintoimprovingtheeffectivenessofprogramsdesignedtopreventandtreatthisdisease.
The goals of the IAS are several fold. Above all, the IAS aims to fostercommunication worldwide among researchers, educators, and health careprofessionals in the field of atherosclerosis and cardiometabolic disorders. Theorganization upholds high quality research and provides a forum forcommunicationofthisresearchamongtheleadinginvestigatorsinmanycountries.Another important aim is to provide educational opportunities for youngerresearchersandhealthcareprofessionals.TheIASis inagrowthphaseandnowaimstoexpanditsactivitiestootherareasthatwillachieveabroaderagenda,suchasnewapproaches toguidelinedevelopmentand increasing theawarenessandtheexchangeofideasanddatatoimproveaccesstocareforpatientswithFHandseverelipoproteindisorders.Havingachievedmanyofitsgoals,wehavelaunchedseveralinitiativestoexpandonthosegoalsandtocreateanewenvironmentforthose in the field of prevention and treatment of atherosclerosis andcardiometabolicdiseases.
The IAS isable topromote itsmissionandobjectives fromamore internationalperspective than other organizations due to its unique position throughout theworldasafederationofmanyregionalandnationalmembers,includingnewandemergingcountrieswithsubstantivelydifferentcultures,environments,genetics,anddiseasesand is thusabletoopenthefieldtonewideasand interpretationsfrombothdevelopedandlesser-developedareasoftheworld.Concepts,medicine,andpeopleareabletobridgeinbothdirections,toenrichtheworld.
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OmanSocietyofLipidandAtherosclerosis(OSLA)shalladvocateforqualitypatientconcerning lipid disorders and atherosclerosis through education, researchpromotion,developmentandapplicationofstandardsandguidelinestoinfluencehealthcarepolicy.In-linewiththemissionstatement,theOSLA‘sobjectivesare:ImprovementaswellasadvancementofPatientCare-OSLAwillaugmentmedicalunderstanding, clinical expertise, and allied occupational activities that deliverimproved and effective patient outcomes.OSLA also aims to encourage patientinvolvement and understanding of lipid related disorders and atherosclerosisevolvingtoupkeepguidelinebaseddiseasecareandprevention.Servicewellnessprograms with sound cardiovascular health information extend the patient-physicianprofessionalrelationship.PromotionofMultidisciplinaryTranslationalResearch(MTR)-OSLAaimstoendorsetranslational research aims, to convert laboratory discoveries in manifolddisciplines into therapeutic gains for patients. This concept débuted MTR, is amultifacetedprocess and isoftenelaboratenecessitating colossal investment intime,moneyandexpertise.OSLAaimstoaugmentMTRbyadvocatingforincreasedresearch resources to support translational lipid research from fundingagenciessuchasthegrantdisbursementoffice(s)atdifferentacademic/medicalinstitutionsandGovernmentalgrantorganizations..
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In 1999 the GCC HealthMinisters meeting in Geneva formed a “Heart diseasecommittee” composed of GCC cardiologists and requested me to chair it. Thecommitteehadits1stmeetinginDohainFebruary2000andplannedalargeGCCCardiovascularSymposiuminDoha.In the year 2001, the GCC Health Ministers approved the committee’srecommendations including the proposed symposium in Doha, Qatar. So, DohahostedthefirstGCCCardiovascularSymposiumonJanuary15–17,2002
That historic conference provided an opportunity for Gulf cardiologists,cardiovascularsurgeons,andothercardiovascularspecialistsintheGCCstatestomeet, exchange ideas and build bridges for scientific andmedical cooperation.During thatmeeting,we proposed to establish a professional societywhichwecalled Gulf Heart Association (GHA). The proposal was unanimously andenthusiasticallyendorsedbytheentiregroupofcardiovascularspecialistspresentduringthatmeetingandsotheGHAwasbornonJanuary16,2002.OverthreeyearstheGHAbecamealandmarkaccomplishmentfortheGCCstates.
ThemainaimofGHA is to improvethequalityofcardiaccare intheGCCstatesthroughitsvariousactivities.
Themajoraimsofourassociationandwhatwehopetoachievearethefollowing:1.RaisingthestandardofcardiaccareintheGCCstates.2.Conductscientificconferencesandsymposia.3.Carryonscientificresearchoncardiovasculardiseases4.Publishprofessionalperiodicalsand information. (GHAhasHeartViewsas itsofficialjournal).5.Createprofessional,educational,andsocialtiesamongmembersoftheGHA.6.Createlinksandcooperationlocallyandwithinternationalmedicalinstitutionsandprofessionalsocieties.7.TheGHAhopestoestablishcriteriaforGCCcardiovascularspecialiststomeethighstandardsofcompetenceandexpertise.8. The GHA seeks to suggest laws to be adopted by the GCC countries for thepreventionofcardiacdiseaseandadvancethecareofpatientswithheartdisease.
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Emirates Cardiac Society (ECS) is a non-profit organization comprising ofcardiologistswithintheUAEthatworkundertheumbrellaoftheEmiratesMedicalAssociation.ECS isstrivingto improvecardiovascularhealththrougheducation,researchandquality patient care. ECS helps people understand the importance of healthylifestyle choices and provides science-based treatment guidelines to healthcareprofessionalstohelpthemprovidequalitycaretotheirpatients.VISION:Toestablishastrongnetworkforthemanagement,educationandresearchsoastoimprovethequalityoflifeandlongevity,throughbetterprevention,diagnosisandtreatmentofheartdisease.MISSION:To educate the public and healthcare professionals in reducing the burden ofcardiovasculardiseaseintheUAE.TheEmiratesCardiacSocietypromoteshealtheducationwithinthecommunity,raisingawarenessofhealthissuesamongstthepopulation and establishes strong partnerships with national and internationalhealthagenciesandestablishments.VALUES:Excellence: The Emirates Cardiac Society is flexible and is able to deal with theexpectationsofthemembersaswellasthepublicandexceedstheirexpectationsby answering queries as well as provides solutions for the problems.Quality: Complieswithbest standards andpractices, ensuring that theEmiratesCardiacSocietywinsthetrustofthemembersandthepublic.TeamWork:TheEmiratesCardiacSocietyactivelyengageswithitsmembersandthepublicandprovidessupportandadvicetothecommunityatlarge.Teamspiritprevails and channels are always open for communication.Financial Effectiveness: Optimal use of resources through good planning, andprioritysettings.
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SCIENTIFIC AGENDA
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DAY1:14THOFOCTOBER2017Defining,identifyingandmanagingseverefamilialhypercholesterolemia09.00 09.10 Welcome Dr.KhalidAl-Rasadi Oman Dr.RaulSantos Brazil 09:10 09:35 LipidmetabolisminFH 09:35 09:45 Dr.KhalidAl-Rasadi OmanDiscussion 09:45 10:10 DiagnosisandnaturalhistoryofhomozygousFH
Dr.Hani Sabour
10:10 10:20 UAEDiscussion
10:20 10:45 ThegeneticsofFH:makingthingssimple 10:45 10:55 Dr.FahadAlzadjali OmanDiscussion
COFFEEBREAK 11:15 11:40 Familyhistory,consanguinityandcascadescreening:whataretheimplications? Dr.FahadAlzadjali Oman11:40 11:50 Discussion 11:50 12:15 DiagnosisandmanagementofpediatricFH
Dr.KhalidAl-Rasadi Oman12:15 12:25 Discussion
LUNCH
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14:00 14:25 DefiningthesevereFHphenotype:ConsensusfromtheInternationalAtherosclerosisSociety Dr.RaulSantos Brazil14:25 14:35 Discussion 14:35 15:00 TreatmentofsevereFHForms1:Theroleofstatinsandezetimibe
Dr.Raul Santos Brazil15:00 15:10 Discussion 15:10 15:35 TreatmentofsevereFHForms2:PCSK9inhibitors
DrKhalidAl-Waili Oman15:35 15:45 Discussion 15:45 16:10 TreatmentofsevereFHForms3:LomitapideandMipomersen Dr.RaulSantos Brazil16:10 16:20 Discussion 16:20 16:45 TreatmentofsevereFHForms4:LDLapheresis
DrKhalidAl-Waili Oman16:45 16:55 Discussion 16:55 17:20 ManagementalgorithmforsevereFH:whatdotheguidelinessay?
DrHaniSabour UAE17:20 17:30 Discussion
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DAY2:15THOFOCTOBER2017SevereFamilialHypercholesterolemiaCaseDiscussions
09:00 09:10 Day2Objectives DrKhalidAl-Waili Oman 09:10 10:10 Cases1:GROUPAPharmacologicaltreatmentofsevereFH
Dr.RaulSantos Brazil
Dr.KhalidAlWaili Oman 09:10 10:10 Cases2:GROUPBCasesonLDLapheresis
Dr.KhalidAl-Rasadi Oman
COFFEEBREAK
10:25 11:25 Cases1:GROUPBPharmacologicaltreatmentofsevereFH
Dr.RaulSantos Brazil
Dr.KhalidAlWaili Oman Dr.FahadAlzadjali Oman
10:25 11:25 Cases2:GROUPACasesonLDLapheresis
Dr.KhalidAl-Rasadi Oman
Dr.HaniSabbour UAE
11:25 12:00 Discussionandwrapup
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ABSTRACTS
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Dr.KhalidAl-Rasadi(Oman)Lipsmetabolisminfamilialhypercholesterolemia Themeatbolismofplasma low-density lipoprotein (LDL-C) is tightly regulated inhumanstodistributecholesterolthroughthecirculatorysystemandintocellsthatrequire extracellular cholesterol. Cholesterol absorption is achieved throughpassage across brush bordermembranes and into intestinal enterocytes in thejejunumthroughtheNiemann-PickC1-Like1(NPC1L1)transportprotein.Ontheother hand ATP binding cassette G5 and G8 proteins (ABCG5/8) appear tonegatively regulate cholesterol transport into enterocytes. Mutations in theABCG5/G8 genes are associated with sitosterolemia, which is characterized byincreasedabsorptionofplantsterols.Cholesterolistransportedfromtheliverintothe circulation by very low density lipoprotein (VLDL) which undergo furthermetabolismtoLDLandthenclearedbytheliverthroughtheLDLreceptors.Familialhypercholesterolaemia(FH) isgeneticdefect intheLDLreceptors(LDLR)whichcanleadtothelif longaccumaltionofLDL-C intheplasmaandeventuallyleadtoprematurecoronaryheartdisease(CHD).Proproteinconvertasesubtilisin/kexintype9(PCSK9)isanotherakeynewplayerin cholesterol homeostasis. PCSK9, by direct interaction with the hepatic LDLR,enhancesitsdegradationbytargetingitfordestructioninthelysosome,disallowingitsnaturalrecyclingloop.Thus,Gain-of-functionmutationsinPCSK9areacauseofhighplasmaLDL-CandLoss-of-functionmutationscan lead to lowplasmaLDL-Candmarkedlyreducedcardiovascularrisk.References1.Marais, A. D. (2004). Familial Hypercholesterolaemia. The Clinical BiochemistReviews,25(1),49–68.2.Shapiro,MichaelD.,andSergioFazio.“PCSK9andAtherosclerosis-LipidsandBeyond.”JournalofAtherosclerosisandThrombosis24.5(2017):462–472.PMC.Web.8Sept.2017.
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Dr.NasreenAL-Sayed(Bahrain) Diagnosis and natural history of homozygous familialhypercholesterolemia Familialhypercholesterolemia(FH)isageneticdisorderoflipoproteinmetabolismresultinginelevatedserumlow-densitylipoprotein(LDL)cholesterollevelsleadingtoincreasedriskforprematurecardiovasculardiseases(CVDs).Thediagnosisofthisconditionisbasedonclinicalfeatures,familyhistory,andelevatedLDL-cholesterollevels aided more recently by genetic testing. As the atherosclerotic burden isdependentonthedegreeanddurationofexposuretoraisedLDL-cholesterollevels,earlydiagnosisandinitiationoftreatmentisparamount.Thistalkdiscussesindepththe clinical features, diagnostic criteria and natural history of familialhypercholesterolemia.Dr.FahadAlzadjali(Oman) Familyhistory,consanguinityandcascadescreening:whataretheimplications?DatafromDutchLipidClinicNetworksuggestthattheprevalenceofheterozygousfamilialhypercholestrolemia(HeFH)highas1in200.Familialhypercholestrolemia(FH)isunderdiagnosed inmost countries specially thosewithhigh consanguinityrate like Arabian Gulf. HeFH is caused either by heterozygous loss-of- functionmutations in lowdensity lipoproteinreceptor (LDLR),heterozygousmutations inapolipoprotein B (APOB) that affect the LDL receptor-binding domain ofapolipoprotein B, or heterozygous gain-of-function mutations in proproteinconvertasesubtilisin/kexintype9(PCSK9).HeterozygousLDLR,APOB,andPCSK9mutationsarefoundin>90,≈5,and≈1%,respectively,ofheterozygousFHsubjectswithacausativemutation.Other raremutationoccurses inautosomal recessivehypercholesterolemia(ARH)genesalsocalledLDLRadaptedprotein(LDLRP1).Cascade genetic screening program to trace familymembers with FH has beensuccessfullyimplementedbycertainpopulationspeciallyintheNetehrlands.Withcascadescreening,identifiedcasesreceivedearlycholestoerolloweringtherapyinwhich year follow up showed a dramatic improvement of their survival andthereforeaverycost-effectivestrategy.
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Dr.KhalidAl-Rasadi(Oman) Diagnosis and management of pediatric familialhypercholesterolemia Therearepotentiallyasmanyas4.5millionindividualsinEuropewithheterozygousfamilial hypercholesterolemia (HeFH)andprobably35millionworldwide (Figure1B),ofwhom20–25%arechildrenandadolescents.ChildrenwithuntreatedHeFHhaveadramaticincreaseinriskofprematureCHDafterage20years.IndividualswithhomozygousFH(HoFH)areatextremelyhighriskand,ifuntreated,manywillmanifestcoronaryorothercardiovasculardiseaseinchildhoodoradolescence.FHisdiagnosedeitheronphenotypiccriteria,involvinganelevatedplasmalowdensitylipoprotein cholesterol (LDL-C) level plus a family history of elevated LDL-C,prematurecoronaryheartdisease(CHD),and/orgeneticdiagnosis,orwithgenetictesting.AchildofaparentwithFHhasa50%probabilityofinheritingFH,therebyemphasizingtheimportanceofafamilypedigreetoidentifyrelativesforscreening.StatinsaresafeandeffectiveinloweringLDL-Cinchildren,15restoreendothelialfunction,andregress thickeningof the intimaof thevesselwallatayoungage.Simvastatin, lovastatin,atorvastatin,pravastatin,fluvastatinandrosuvastatinareapprovedintheUSAandEuropeforuseinchildrenwithFH.Ezetimibeisapprovedforusefromage10yearsintheUSAandEurope,andisverywelltoleratedwithminimalsideeffects.Thebile-acidsequestrantscausegastrointestinalsideeffects;colesevelamisthebesttoleratedoftheseagentsandisapprovedintheUSAfromage 10 years although not in Europe. For HoFH treatment with a statin andezetimibemustbestartedatdiagnosis.Ifavailable,lipoproteinapheresisshouldbestarted as soon as technically possible; this may be as early as age 2 years inspecialized centers. Two new agents, oral lomitapide, amicrosomal triglyceridetransferproteininhibitor,andinjectablemipomersen,anantisenseRNAtherapy,both of which target hepatic production of atherogenic apoB-containinglipoproteins,were recentlyapproved in theUSAasadjunct therapy forHoFH inpatientsaged≥18and≥12years,respectively.Ofnoveltherapiesindevelopment,monoclonalantibodytherapiestoPCSK9(alirocumabandevolocumab)showthemostpromise, loweringbothLDL-CandLp(a),althoughpatientshomozygousforLDLR-nullmutations showedapoor therapeutic response,asexpected fromthemechanismofaction.Paediatrictrialsoftheseagentsareunderwayorplanned.
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References1. European Atherosclerosis Society Consensus Panel. Familialhypercholesterolaemia in children and adolescents: gaining decades of life byoptimizingdetectionandtreatment.EurHeartJ.2015Sep21;36(36):2425-37.2.ClinicalguidancefromtheNationalLipidAssociationExpertPanelonFamilialHypercholesterolemia.FamilialHypercholesterolemia:Screening,diagnosisandmanagementofpediatricandadultpatients.JournalofClinicalLipidology(2011)5,S1–S8.Dr.RaulSantos(Brazil) Defining the severe familial hypercholesterolemia phenotype:ConsensusfromtheInternationalAtherosclerosisSociety(IAS) Familialhypercholesterolemia(FH) isassociatedwithanelevated lifetimeriskofatheroscleroticcardiovasculardisease(ASCVD)however,thereisheterogeneityonthisrisk.ClassicallyFHisdividedinheterozygous(He)andhomozygous(Ho)formsandtheformerpresentasignificantlyhigherrisknotonlyofASCVDbutalsoaorticvalvular and supra-valvular disease. Recent evidence has shown that there isoverlaponthephenotypicmanifestationsbetweenHeandHoFHformsandthisfact bears prognostic implications. The IAS therefore developed a consensusdocumentinordertobetteridentifyhigherriskFHpatientsconsideringthegreaterunmet needs for LDL-cholesterol reduction on this population and the highmonetarycostofavailabletherapiesforthesemoresevereformsofFH.SevereFHisdefinednotonlybasedonveryhighLDL-Cbloodlevelsbutalsoonthepresenceof clinical ASVCD, severe burden of coronary subclinical atherosclerosis andassociationof risk factors like smoking, diabetes, lowHDL-C, hypertension, highLipoprotein(a)levelsandrenalfailureamongothers.ThosehigherriskindividualswhodonotattainrecommendedLDL-cholesterolgoalsarethenaturalcandidatesfor PCSK9 inhibitors, lomitapide and mipomersen (in the case of HOFH) andlipoproteinapheresis.Reference:SantosRDetal.LancetDiabetesEndocrinol.2016;4:850-61
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Dr.NasreenAL-Sayed(Bahrain) Treatment of severe familial hypercholesterolemia forms 1: Theroleofstatinsandezetimibe Statins are presently themainstay in themanagement of FH patients, althoughnewerdrugs,LDLapheresis,andotherinvestigationaltherapiesmayplayaroleincertain subsets of FH, which are challenging to treat. Together these noveltreatments have notably improved the prognosis of FH, especially that of theheterozygouspatients.Despitetheseachievements,amajorityofpatientsfailtoattain targeted lipid goals owing to persistent shortcomings in diagnosis,monitoring, and treatment. This talk aims to highlight goals of therapy, andmanagementoptions inpatientswithFHwith focusonstatinsandezetimibeasagentsofchoice.DrKhalidAl-Waili(Oman)Treatment of severe Familial Hypercholesterolemia: PCSK9inhibitors Hyperlipidemia is a well-established risk factor for developing cardiovasculardisease (CVD). The recent American College of Cardiology and American HeartAssociationguidelinesonlipidmanagementemphasizetreatmentofindividualsatincreased risk for developing CVD events with 3-hydroxy-3-methylglutarylcoenzymeAreductaseinhibitors(statins)atdosesproventoreduceCVDevents.However,therearelimitedoptionsforpatientswhoareeitherintoleranttostatintherapy,developCVDdespitebeingonmaximallytoleratedstatintherapy,orhavesevere hypercholesterolemia. Recently the Food and Drug Administrationapproved two novel medications for low-density lipoprotein (LDL)-cholesterolreduction: Evolocumab and Alirocumab. These agents target and inactivateproprotein convertase subtilsin-kexin type 9 (PCSK9), a hepatic protease thatattaches and internalizes LDL receptors into lysosomes hence promoting theirdestruction.BypreventingLDLreceptordestruction,LDL-Clevelscanbelowered50%-60%abovethatachievedbystatintherapyalone.The addition of evolocumab to statin therapy over several years significantlyreducedcardiovascularmorbidityandmortalityinpatientswithclinicallyevidentatheroscleroticcardiovasculardisease,accordingtoresultsfromtheFOURIERtrial.
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Dr.RaulSantos(Brazil) Treatment of severe familial hypercholesterolemia forms 2:LomitapideandMipomersen Lomitapideandmipomersenare2medicationsapproved for LDL-C reduction inhomozygous familial hypercholesterolemia. Lomitapide reduces VLDL andchylomicronproductionbyinhibitingthemicrosomaltriglyceridetransferprotein(MTP)respectivelyintheliverandthegut.Therefore,lomitapidecanreducebloodLDL-Cbyup to50%.Due to itsuniqueactionmechanism lomitapidecan inducereductioninfatabsorptionintheintestineandinduceliversteatosis.Recentofanopen label long-term extension study (mean duration 5 years) as shown thatlomitapidemaintains its lipid loweringefficacywithgoodtolerabilityhowever, itincreased the amount of liver fat. During the extension trial, 21.1% patientsexperienceda≥5×upper limitofnormal increaseinalanineaminotransferaseoraspartate aminotransferase. Increases ≥5× upper limit of normal were typicallyassociated with concomitant use of cytochrome P450 3A4 inhibitors or excessalcoholuse.However,theywereeasilymanaged.Mipomersenisasecond-generationantisenseoligonucleotide(ASO)thatbindstoapolipoprotein B (apoB) messenger RNA and therefore blocks translation.MipomersenreducesVLDL,LDLandLp(a)concentrationsbyaround25%.Arecentstudysuggeststhatmipomersencanreducecardiovasculareventsinseverefamilialhypercholesterolemiapatients.However,thetolerabilityofthismedicationisverylowduetoinjectionsitereactionsandflu-likesymptoms.Mipomeresen,canalsoinduceliverfataccumulationandraiseliverenzymes.Bothdrugscanbeusedonpatientsundergoinglipoproteinapheresis.References:BlomDJetal.Circulation.2017;136(3):332-335CuchelMetal.Lancet.2013;381(9860):40-6.DuellPBetal.JClinLipidol.2016;10:1011-21SantosRDetal.EurHeartJ.2015;36:566-75
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DrKhalidAl-Waili(Oman)
TreatmentofsevereFamilialHypercholesterolemia:LDLApheresis Familial hypercholesterolemia (FH) is an autosomal co-dominant disordercharacterized by a marked elevation of serum low-density lipoprotein (LDL)cholesterol (LDL-C) concentration, which in turn is associated with a greatlyincreased risk of premature cardiovascular disease. International consensusrecommendstheuseofstatinsasthefirstlineoftreatmentforpatientswiththiscondition. However, homozygote FH patients with persistently elevated LDL-Clevelsareusuallyresistanttomultiple-drugtherapy.Fortunately,LDLapheresis(orsimply ‘lipoproteinapheresis’)providesa treatmentoption forpatientswhoarerefractory or intolerant to lipid- loweringmedications, or if there is progressivecardiovasculardiseasedespitemaximaldrugtherapy.Lipoprotein apheresis is an extracorporeal LDL-C-lowering treatment similar inconcept to renal dialysis. There are now five main methods for extracorporeallipoprotein apheresis in use, namely dextran sulfate adsorption (DSA), heparinextracorporealLDLprecipitation(HELP),polyacrylatefullbloodadsorption(PFBAor DALI system) using hemoperfusion, immunoadsorption, and filtrationplasmapheresis.Thehighcostandinvasivenatureoflipoproteinapheresislimitsuptake;however,it is an important treatment modality that should be considered in carefullyselectedpatients.Atpresent, lipoproteinapheresis,combinedwithhigh-dosestatinandezetimibetherapy, is the best availablemeans of treating patients with homozygous andstatin-refractoryheterozygousfamilialhypercholesterolaemia(FH).
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DrHaniSabour(UAE) ManagementalgorithmforsevereFHwhatdotheguidelinessay? SeverefamilialhyperlipidemiacanberecognizedbyparticularlyhighlevelsofLDL-Cintherangeof5–10mmol/L(_200–400mg/dL)inadulthood.GenerallyTGlevelsarenormal,butcanoccasionallyberaisedinadults,particularlyiftheyareobese.ThetypicalHeFHpatientmaynot inappearanceconformatall to theclinician’sconcept of a coronary-prone individual. CVD risk estimationmethods based onmultivariate risk equations alone are not sufficient to estimate the risk ofindividuals with FH. Furthermore, the risk related to HeFH can be substantiallyameliorated by early treatment. Untreated, the majority of affected men andwomenwillhavesymptomaticcoronarydiseaseby60yearsandhalfofthemenand15%ofthewomenwillhavedied.Ontheotherhand,patientswhostartattendingalipidclinicbeforetheydevelopclinicalCADmayenjoyanormallifeexpectancyifwellmanaged.It cannot be overemphasized that the management of HeFH does not simplyinvolveadviceaboutahealthylifestyleandtheprescriptionoflipid-loweringdrugs,butalso involvesensuring thatpatientshavepromptaccess to investigations todetectthepresenceofsignificantatherothromboticdisease.IdeallymanagementofHeFHshouldinvolvealipidclinic.Lifestyleadvice,particularlyaboutdietandtheavoidanceofsmoking,isimportantinHeFH.HoFH is always an extremely serious disease, which untreated leads to deathtypicallyinadolescenceorearlyadulthoodduetomyocardialischaemiaoraorticstenosis.TheworstprognosisoccurswhenbothmutationsleadtocompletefailureofexpressionofLDLRratherthantodefectiveLDLRexpression.Patients with suspected HoFH are promptly referred to specialist centres for acomprehensiveACVDevaluationandclinicalmanagement. Lifestyle interventionandmaximalstatintherapyarethemainstaysoftreatment,ideallystartedinthefirst year of life or at an initial diagnosis, oftenwith ezetimibe and other lipid-modifyingtherapy.AspatientsrarelyachieveLDL-Ctargets,adjunctivelipoproteinapheresisisrecommendedwhereavailable,preferablystartedbyage5andnolaterthan 8 years. The number of therapeutic approaches has increased followingapprovaloflomitapideandmipomersenforHoFH.GiventheseverityofACVD,werecommendregularfollow-up,includingDopplerechocardiographicevaluationof
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theheartandaortaannually,stresstestingand,ifavailable,computedtomographycoronaryangiographyevery5years,orlessifdeemednecessary.Wewillreviewtheguidelinesemphasizingtheneedandmethodsofscreeningandassessment for atherosclerosis that are required in these patients and stepwiseapproachtoearlytreatmentandspecializedlipidclinics.
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FACULTY GUESTS
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FahadAl-ZadjaliCollegeofMedicine&HealthScienceSultanQaboosUniversityMuscat,[email protected]
Dr. Fahad AL Zadjali has obtained PhD degree inmolecular endocrinology in2011. Currently Dr. Al-Zadjali is an assistant professor at Department ofBiochemistry ,College of Medicine & Health Science, at the Sultan QaboosUniversity. Dr Al-Zadjali research focus is on proteomic profiling of cellularproteins involved inmodulationofhormonalsensitivity.Research involvedonroleofGrowthhormoneondiseasemodulationinnon-alcoholicsteatohepatitisand inflammatory bowl disease. Hormonal therapy in thymic rejuvenation inelderly subjects undergoing immunization and patients undergoingchemotherapy.DrAlZadjalihasexpertiseincomplexheritabilitystudiesonmetabolicsyndromeandstudylipidandbloodglucosevariabilityinextendedfamiliesusingmeasuredgenotypeanalysis.Similalry,analysismonogenicdisordersusingnextgenerationsequencing.Technicalexpertiseoncomplexgenomicdataanalysis,proteomicanalysis, Laboratory animal microsurgery , generation of genetic modifiedanimals.Dr.FahadisanationalmemberofsciencecommissionatUNESCO.DrFahadisafocalpointcollaboratorbetweenSultanQaboosUniversityandotherEuropeanandAmericanUniversities.
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HaniSabbourConsultantCardiologistandElectrophysiologistSheikhKhalifaMedicalCityAbuDhabi,[email protected]
HaniM.SabbouriscurrentlyConsultantCardiologistandElectrophysiologistatSKMCandalsoformerInternalMedicineResidencyProgramDirectoratAlAinHospital,AlAin,UnitedArabEmirates.HeservedasDirectorofCardiacArrhythmiaandElectrophysiologyatLandmarkMedicalCenterinRhodeIslandUSAfrom2003until2012.Since January2013,hehasheldanacademicappointmentasClinicalAssistantProfessor Cardiovascular Disease atWarren Alpert School ofMedicine, BrownUniversity.HeestablishedacomprehensivePulmonaryHypertensionCenterandPH registry at SKMC aswell as leading the SEHAPH guidelinewriting team tocompletetheSEHAclinicalpracticeguidelinesinPAH.Dr Sabbour received his medical training at the Faculty of Medicine, KuwaitUniversity,culminatinginamedicaldoctoratein1994andawardedHisHighnessThe Emir's Gold Medal with First Class Honors . He completed residencies atKuwaitInstituteforMedicalSpecializationsandSUNYStonyBrook–EastMeadowCampusin1995and1998,respectively.HewasalsoClinicalandResearchFellowinclinicalcardiacelectrophysiologyatMassachusettsGeneralHospital–HarvardMedical School, where he received ABIM Clinical Cardiac ElectrophysiologyCertification in 2003. He is ABIM certified in Internalmedicine, CardiovascularDisease , Cardiac Electrophysiology, Advanced Heart Failure and CardiacTransplant , ECHO and Nuclear Cardiology . Dr Sabbour has been involved inmultipleclinicaltrials;hisextensiveresearchoncardiovasculardiseaseshasbeenpublished in a number of industry-related publications. His clinical interestsinclude integrateddevicetherapyandCHF,correlativecardiac imaging,nuclearcardiology,arrhythmiamanagementparticularlyatrialfibrillation,managementofvalvularheartdisease,drugmonitoringandmanagementprogramandetc.Dr.Sabbour is currently a Fellow of the American College of Cardiology , HeartRhythmSocietyandtheAmericanCollegeofPhysicians,andEgyptianAmericanMedicalAssociation.
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KhalidAl-RasadiSeniorConsultantBiochemist/LipidologistPresidentofOmanSocietyofLipid&AtherosclerosisHeadofDepartmentofBiochemistryDepartmentofClinicalBiochemistrySultanQaboosUniversityHospitalMuscat,[email protected]@squ.edu.om
Dr.KhalidAlRasadiwasborninOmanwherehestudiedmedicineandobtainedhisMedicalDegreein1998.Dr.AlRasadigothispostgraduatetraininginMedicalBiochemistryatMcgillUniversityinCanadaduringtheperiodof2002-2006.Hespentanother twoyears inCanada,workingas scientist at theRoyalVictoriaHospital,McgillUniversity,underthedirectsupervisionofProf.JacquesGenest.Therehewasabletoperformanumberofstudiesonthemoleculargeneticsoffamilialhypercholesterolemiaandhypoalphalipoproteinemia.InthatperiodDr.Al Rasadi also received strong training at the lipid clinic of theRoyal VictoriaHospital, Mcgill University. He was also awarded a certificate in ClinicalLipidologybytheNationalLipidAssociationintheUnitedStatein2008.Dr.AlRasadiistheHeadofDepartmentofBiochemistryattheSultanQaboosUniversityHospitalinOman.Dr.AlRasadiisoneofthefoundermembersoftheOmanSocietyofLipid&Atherosclerosis(OSLA),andsincethenhecontributedsignificantly in the recognition of OSLA as an active society nationally andinternationally as key player in promoting scientific education and generalawareness relating to lipid disorders and atherosclerosis through organizingconferencesdeliveredbyveritableexpertsinthefieldoflipidresearch,aswellaspublicoutreachactivities.Dr.AlRasadiisamemberofnationalandinternationalmedicalassociationsandamemberofOmanMedicalJournalandBBA-Clinicaljournaleditorialboard.Hehas32publicationsand1chapterbook(totalCitations156,H-Index:8).
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KhalidAl-WailiSeniorConsultantSultanQaboosUniversityHospitalMuscat,[email protected]
Dr.KhalidAlWailiwasborninOman.HestudiedmedicineinOmanandobtainedhisMedicalDegreein2001.Dr.AlWailigothistraininginMedicalBiochemistryatMcGillUniversityinCanada2009.ThenhespenttwoyearsinCanada,workingas a fellow and Scientist in clinical Lipidology at the Royal Victoria Hospital,UniversityofCanada,underthedirectsupervisionofProf.JacquesGenest,MD.Therehewasabletoperformanumberofstudiesonthemoleculargeneticsoffamilialhypercholesterolemiaandhypoalphalipoproteinemia.InthatperiodDr.AlWaili also received strong training in the Lipid Clinic of the Royal VictoriaHospital University of McGill, under the direct supervision of Prof. JacquesGenest,MDfrom2009-2010.TheNationalLipidAssociationintheUnitedStatesalsoawardedhimacertificateinClinicalLipidologyin2010.Dr. Al Waili is a Senior Consultant at the Sultan Qaboos University Hospital(SQUH)andheistheDeputyHeadofDepartmentofBiochemistryattheSultanQaboosUniversityHospital(SQUH)inOman.Moreover,heisamemberoftheUnit of Lipid and LDL-Apheresis at SQUH.Dr. AlWaili is a founder of Lipid&Atherosclerosis (OSLA) in 2012. In addition, he was trying to improve therecognitionofOSLAasanactivesocietynationallyandinternationallyasakeyplayerinpromotingscientificeducationandgeneralawarenessrelatingtolipiddisordersandatherosclerosisthroughmini-conferences,organizationofplenarylecturesdeliveredbyveritableexpertsinthefieldofLipidresearch,aswellaspublicoutreachactivities.Dr.AlWailiisamemberofnationalandinternationalmedicalassociations.Hehas several publication and one chapter book in the field of lipid andatherosclerosis.
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NasreenAL-SayedMedicalDirectorConsultantEndocrinologist&LipidologistGulfDiabetesSpecialistCenterManama,[email protected]
Dr. Al-Sayed graduated from Arabian Gulf University, college of Medicine inBahrain.SheisAmericanBoardCertified.ShecompletedherResidencytrainingin Internalmedicine at Sinai-GraceHospital,Wayne StateUniversity, Detroit,Michigan, USA and Fellowship training in Endocrinology and Diabetes atCleveland Clinic Foundation, Ohio, USA. Dr. Al-Sayed is also American BoardCertifiedinClinicalLipidologyandafellowofAmericanCollegeofPhysiciansandfellow of American College of Endocrinologists. She has worked as assistantprofessoratArabianGulfUniversityandiscurrentlytheMedicalDirectoratGulfDiabetesSpecialistCenterinBahrain.Dr.Al-SayedisamemberoftheNationalLipidAssociationandotherDiabetesandEndocrineAssociationsandhasseveralresearchpublications.She isamemberofBoardofDirectorsoftheAmericanAssociation of Clinical Endocrinologist, Gulf Chapter and amember of expertpanel for Middle East Familial Hypercholesterolemia guidelines and FH Gulfregistry.SheisthenationalleadinvestigatorforFHinBahrain.Dr. Alsayed participated with expert panel and published first consensusrecommendation for management of plasma lipid disorders for Middle East2016.
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RaulD.SantosDirectorLipidClinicHeartInstitute(InCor)UniversityofSãoPauloMedicalSchoolHospitalSãoPaulo,[email protected]@incor.usp.br
RaulD.Santos,MD,MSC,PhD,isDirectoroftheLipidClinicoftheHeartInstitute(InCor)of theUniversityof SãoPaulo,Brazil.He is associateprofessorof theCardiopulmonaryDiseaseDepartment at theUniversity of São Paulomedicalschool and one of the coordinators of the “Hipercol Brasil” FamilialHypercholesterolemiaGeneticCascadeScreeningProgram.HeisalsoScientificAdvisor of the Preventive Medicine Centre and Cardiology Program of theHospital IsraelitaAlbertEinstein inSãoPaulo.After completinghis training incardiologyDr.SantosobtainedmasterandPHDdegreesinfoodscienceattheUniversityofSaoPaulo.Hisresearchinterestsincludelipidmetabolism,severeformsofgeneticdyslipidemias,especiallyfamilialhypercholesterolemiaandHDLdeficiency, non-alcoholic fatty liver disease and imaging in atherosclerosisespeciallydetectionofvascularcalcification.Hehaspublishedmorethan200papers in peer-review journals like the Lancet, The New England Journal ofMedicine,Atherosclerosis,ATVB,Circulation,TheEuropeanHeartJournal,JACC,JournalofLipidResearch,LancetDiabetes&EndocrinologyandCurrentOpinionofLipidologyamongothers.Dr.SantoshascoordinatedandparticipatedinmanyBrazilian and International guidelines on dyslipidemia, familialhypercholesterolemia, atherosclerosis prevention, metabolic syndrome,obesity,diabetesandcoronaryheartdisease.Heisthecurrentscientificdirector(2016-2017)oftheBrazilianSocietyofCardiology(SBC),presidentelect(2019-2021) of the International Atherosclerosis Society (IAS), a member of theConsensusPanelonDyslipidemiasoftheEuropeanAtherosclerosisSociety(EAS)and is the vice president of the Iberoamerican FamilialHypercholesterolemianetwork (IBAFHN). In addition, he is one of the associate editors ofAtherosclerosis, theJournalofClinicalLipidologyandtheEuropeanJournalofPreventiveCardiology.
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WaelAbdulrahmanAlmahmeedConsultantCardiologistandChiefofCardiologyInstituteofCardiacSciencesSheikhKhalifaMedicalCityAbuDhabi,[email protected]@gmail.com
DrWaelAbdulrahmanAlmahmeedisaConsultantCardiologistattheHeartandVascular Institute inClevelandClinicAbuDhabi.He isalsoaClinicalAssociateProfessorattheUAEUniversityandhasanAdjunctStaffpositionatClevelandClinic,Ohio.DrAlmahmeed completedhis undergraduatemedical training at theMedicalSchool in the University of Southampton, England. He continued his postgraduatestudiesinInternalMedicineandCardiologyattheUniversityofBritishColumbia, Vancouver, Canada. This was followed by a fellowship inEchocardiographyatthesameinstitution.HereceivedhisFellowshipof theRoyalCollegeofPhysiciansandSurgeonsofCanadainInternalMedicinein1994andinCardiologyin1995.SubsequentlyhebecameaFellowoftheAmericanCollegeofChestPhysicians(1996),FellowoftheAmericanCollegeofPhysicians (1998), Fellowof theAmericanCollegeofCardiology(1998)andFellowoftheEuropeanSocietyofCardiologyin2004.He is a foundation member of the Gulf Heart Association and the EmiratesCardiacSociety.UnderhisleadershipintheEmiratesCardiacSociety,theSocietywonthebidtohosttheWorldCongressofCardiologyinDubaiin2012,theWorldTobaccoorHealthCongressinAbuDhabiin2015,theAsiaPacificCongressofCardiologyinAbuDhabiin2015andWorldCongressofPediatricCardiologyin2016.HealsoestablishedanannualmeetingfortheEmiratesCardiacSocietyinUAE.HeisthepastpresidentoftheEmiratesCardiacSocietyandtheGulfHeartAssociation. He is the current president Elect of the Asia Pacific Society ofCardiologyandthecurrentGovernorfortheAmericanCollegeofCardiologyGulfChapter.In2000,heinitiatedWorldHeartDayintheUAE.Todayitiscelebratedbymanyinstitutionsacrossthecountry.DuringhistenureasDeputyChiefMedicalOfficer,atSheikhKhalifaMedicalCity(SKMC)hewaschairoftheQualitysteeringcommittee.Underhisleadership,
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SKMCwasoneofthefirsthospitals inAbuDhabitoreceiveJointCommissionInternationalAccreditationin2008.Under his leadership, SKMC was the first center in the Middle East to beaccreditedasaChestPainCenterin2009,andagainin2012.In2012,SKMCEchodepartmentwasaccreditedbytheEuropeanAssociationofEchocardiography.HishospitalwasthefirstoverseascentertoparticipateintheAmericanCollegeofCardiology’sNCDRActionandCathPCIRegistries.In2004,heestablishedthe firstcenter in theMiddleEast thatprovided24/7PrimaryPCI.Followingthat,hisdepartmentbecamethemostcomprehensivecardiac department in the UAE, providing all the necessary services fromElectrophysiology,AdultCongenitalHeartDiseasetoRehabilitation.In2014,hisdepartmentwasawardedasacenterofexcellence,bytheAmericanCollegeofCardiology.In2013,heinitiatedthefirstCardiologyFellowshipProgramintheUAE.AsChairoftheCMEcommittee,attheHealthAuthority,heestablishedmandatoryCMEhours for health care professionals and also established a system for CMEaccreditation.DrAlmahmeed'sresearchinterestsincludeCoronaryArteryDiseasepreventioninthedevelopingcountries,Hypertension,Dyslipidemia,andRegistriesonAcuteCoronarySyndromes,HeartFailureandAtrialFibrillation.HewasthenationalcoordinatorfortheInter-HeartStudy.HewasonthesteeringcommitteeoftheGulf Race, Gulf Coast, Gulf Care and Gulf Safe Registries. He has over 150publications to his credit, andwas the national coordinator for a number ofMulti-CenterTrialsincludingElixa,ExamineandGarfieldTrials.HeiscurrentlyaCo-InvestigatoroftheGulfFHregistryandtheGulfDyspneastudy.DrAlmahmeed'sexpertiseisinnon-invasiveCardiology/EchocardiographyandHeartDiseaseinPregnancy.
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