ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons Robert O. Bonow, Blase A. Carabello, Kanu Chatterjee, Antonio C. de Leon, Jr, David P. Faxon, Michael D. Freed, William H. Gaasch, Bruce Whitney Lytle, Rick A. Nishimura, Patrick T. O’Gara, Robert A. O’Rourke, Catherine M. Otto, Pravin M. Shah, Jack S. Shanewise, Sidney C. Smith, Jr, Alice K. Jacobs, Cynthia D. Adams, Jeffrey L. Anderson, Elliott M. Antman, David P. Faxon, Valentin Fuster, Jonathan L. Halperin, Loren F. Hiratzka, Sharon A. Hunt, Bruce W. Lytle, Rick Nishimura, Richard L. Page and Barbara Riegel J. Am. Coll. Cardiol. 2006;48;1-148 doi:10.1016/j.jacc.2006.05.021 Downloaded from content.onlinejacc.org by on September 1, 2006 This information is current as of September 1, 2006 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://content.onlinejacc.org/cgi/content/full/48/3/e1 Downloaded from content.onlinejacc.org by on September 1, 2006 JournaloftheAmericanCollegeofCardiology Vol.48,No.3,2006 ©2006bytheAmericanCollegeofCardiologyFoundationandtheAmericanHeartAssociation,Inc. ISSN0735-1097/06/$32.00 PublishedbyElsevierInc. doi:10.1016/j.jacc.2006.05.021 ACC/AHA PRACTICE GUIDELINES ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons WRITING COMMITTEE MEMBERS Robert O. Bonow, MD, FACC, FAHA, Chair Blase A. Carabello, MD, FACC, FAHA Bruce Whitney Lytle, MD, FACC Kanu Chatterjee, MB, FACC Rick A. Nishimura, MD, FACC, FAHA Antonio C. de Leon, JR, MD, FACC, FAHA Patrick T. O’Gara, MD, FACC, FAHA David P. Faxon, MD, FACC, FAHA Robert A. O’Rourke, MD, MACC, FAHA Michael D. Freed, MD, FACC, FAHA Catherine M. Otto, MD, FACC, FAHA William H. Gaasch, MD, FACC, FAHA Pravin M. Shah, MD, MACC, FAHA Jack S. Shanewise, MD* *SocietyofCardiovascularAnesthesiologistsRepresentative TASK FORCE MEMBERS Sidney C. Smith, JR, MD, FACC, FAHA, Chair Alice K. Jacobs, MD, FACC, FAHA, Vice-Chair Cynthia D. Adams, MSN, APRN-BC, FAHA Loren F. Hiratzka, MD, FACC, FAHA‡ Jeffrey L. Anderson, MD, FACC, FAHA Sharon A. Hunt, MD, FACC, FAHA Elliott M. Antman, MD, FACC, FAHA† Bruce W. Lytle, MD, FACC, FAHA David P. Faxon, MD, FACC, FAHA‡ Rick Nishimura, MD, FACC, FAHA Valentin Fuster, MD, PHD, FACC, FAHA‡ Richard L. Page, MD, FACC, FAHA Jonathan L. Halperin, MD, FACC, FAHA Barbara Riegel, DNSC, RN, FAHA †ImmediatePastChair;‡FormerTaskForcememberduringthiswritingeffort ThisdocumentwasapprovedbytheAmericanCollegeofCardiologyFoundationBoardofTrusteesinMay2006andbytheAmericanHeartAssociationScienceAdvisoryand CoordinatingCommitteeinMay2006. Whencitingthisdocument,theAmericanCollegeofCardiologyFoundationrequeststhatthefollowingcitationformatbeused:BonowRO,CarabelloBA,ChatterjeeK,deLeon ACJr.,FaxonDP,FreedMD,GaaschWH,LytleBW,NishimuraRA,O’GaraPT,O’RourkeRA,OttoCM,ShahPM,ShanewiseJS.ACC/AHA2006guidelinesforthe managementofpatientswithvalvularheartdisease:areportoftheAmericanCollegeofCardiology/AmericanHeartAssociationTaskForceonPracticeGuidelines(WritingCommittee toDevelopGuidelinesfortheManagementofPatientsWithValvularHeartDisease).AmericanCollegeofCardiologyWebSite.Availableat:http://www.acc.org/clinical/guidelines/ valvular/index.pdf. ThisarticlehasbeencopublishedintheAugust1,2006issueofCirculation. Copies:ThisdocumentisavailableontheWorldWideWebsitesoftheAmericanCollegeofCardiology(www.acc.org)andtheAmericanHeartAssociation(www.my.american- heart.org).Singlecopiesoftheexecutivesummaryareavailablebycalling1-800-253-4636orwritingtheAmericanCollegeofCardiologyFoundation,ResourceCenter,at9111Old GeorgetownRoad,Bethesda,MD20814-1699.Topurchasebulkreprints,fax:212-633-3820,ore-mail:[email protected]. Permissions:Multiplecopies,modification,alteration,enhancement,and/ordistributionofthisdocumentarenotpermittedwithouttheexpresspermissionoftheAmericanHeart Association.Pleasedirectrequeststocopyright.permissions@heart.org. Downloaded from content.onlinejacc.org by on September 1, 2006 e2 Bonowetal. JACCVol.48,No.3,2006 ACC/AHAPracticeGuidelines August1,2006:e1–148 TABLE OF CONTENTS 3.2.3.1. Pathophysiology................................e28 3.2.3.2. NaturalHistory.................................e29 PREAMBLE..............................................................................e4 3.2.3.2.1. AsymptomaticPatients WithNormalLeft 1. INTRODUCTION.............................................................e5 VentricularFunction........e29 1.1. OrganizationoftheCommitteeandEvidence 3.2.3.2.2. AsymptomaticPatients Review..........................................................................e5 WithDepressedSystolic 1.2. ScopeoftheDocument...............................................e6 Function...........................e32 2. GENERALPRINCIPLES................................................e8 3.2.3.2.3. SymptomaticPatients......e32 2.1. EvaluationofthePatientWithaCardiacMurmur...e8 3.2.3.3. DiagnosisandInitialEvaluation......e32 2.1.1. Introduction......................................................e8 3.2.3.4. MedicalTherapy...............................e33 2.1.2. ClassificationofMurmurs................................e8 3.2.3.5. PhysicalActivityandExercise..........e35 2.1.2.1. DynamicCardiacAuscultation...........e9 3.2.3.6. SerialTesting....................................e35 2.1.2.2. OtherPhysicalFindings.....................e9 3.2.3.7. IndicationsforCardiac 2.1.2.3. AssociatedSymptoms.......................e10 Catheterization..................................e36 2.1.3. ElectrocardiographyandChest 3.2.3.8. IndicationsforAorticValveReplacement Roentgenography............................................e11 orAorticValveRepair..........................e37 2.1.4. Echocardiography............................................e11 3.2.3.8.1. SymptomaticPatients 2.1.5. CardiacCatheterization..................................e12 WithNormalLeft 2.1.6. ExerciseTesting..............................................e12 VentricularSystolic 2.1.7. ApproachtothePatient.................................e12 Function...........................e37 2.2. ValveDiseaseSeverityTable.....................................e13 3.2.3.8.2. SymptomaticPatients 2.3. EndocarditisandRheumaticFeverProphylaxis........e13 WithLeftVentricular 2.3.1. EndocarditisProphylaxis.................................e13 Dysfunction......................e37 2.3.2. RheumaticFeverProphylaxis.........................e16 3.2.3.8.3. AsymptomaticPatients....e38 2.3.2.1. GeneralConsiderations.....................e16 3.2.4. ConcomitantAorticRootDisease.................e39 2.3.2.2. PrimaryPrevention...........................e16 3.2.5. EvaluationofPatientsAfterAorticValve 2.3.2.3. SecondaryPrevention........................e17 Replacement....................................................e39 3.2.6. SpecialConsiderationsintheElderly............e40 3. SPECIFICVALVELESIONS.......................................e17 3.3. BicuspidAorticValveWithDilatedAscending 3.1. AorticStenosis...........................................................e17 Aorta...........................................................................e40 3.1.1. Introduction....................................................e17 3.4. MitralStenosis...........................................................e41 3.1.1.1. GradingtheDegreeofStenosis.......e18 3.4.1. PathophysiologyandNaturalHistory............e41 3.1.2. Pathophysiology..............................................e19 3.4.2. IndicationsforEchocardiographyinMitral 3.1.3. NaturalHistory...............................................e20 Stenosis............................................................e42 3.1.4. ManagementoftheAsymptomaticPatient...e20 3.4.3. MedicalTherapy.............................................e45 3.1.4.1. Echocardiography(Imaging, 3.4.3.1. MedicalTherapy:General................e45 Spectral,andColorDoppler)in 3.4.3.2. MedicalTherapy:Atrial AorticStenosis..................................e20 Fibrillation.........................................e45 3.1.4.2. ExerciseTesting................................e22 3.4.3.3. MedicalTherapy:Preventionof 3.1.4.3. SerialEvaluations..............................e22 SystemicEmbolization......................e46 3.1.4.4. MedicalTherapy...............................e22 3.4.4 RecommendationsRegardingPhysicalActivity 3.1.4.5. PhysicalActivityandExercise..........e23 andExercise....................................................e46 3.1.5. IndicationsforCardiacCatheterization.........e23 3.4.5. SerialTesting..................................................e47 3.1.6. Low-Flow/Low-GradientAorticStenosis.....e23 3.4.6. EvaluationoftheSymptomaticPatient.........e47 3.1.7. IndicationsforAorticValveReplacement.....e24 3.4.7. IndicationsforInvasiveHemodynamic 3.1.7.1. SymptomaticPatients.......................e25 Evaluation.......................................................e47 3.1.7.2. AsymptomaticPatients.....................e25 3.4.8. IndicationsforPercutaneousMitralBalloon 3.1.7.3. PatientsUndergoingCoronaryArtery Valvotomy.......................................................e50 BypassorOtherCardiacSurgery.....e26 3.4.9. IndicationsforSurgeryforMitralStenosis....e53 3.1.8. AorticBalloonValvotomy..............................e26 3.4.10. ManagementofPatientsAfterValvotomyor 3.1.9. MedicalTherapyfortheInoperablePatient..e27 Commissurotomy............................................e54 3.1.10. EvaluationAfterAorticValveReplacement..e27 3.4.11. SpecialConsiderations....................................e55 3.1.11. SpecialConsiderationsintheElderly............e27 3.4.11.1. PregnantPatients..............................e55 3.2. AorticRegurgitation..................................................e27 3.4.11.2. OlderPatients...................................e55 3.2.1. Etiology...........................................................e27 3.5. MitralValveProlapse.................................................e55 3.2.2. AcuteAorticRegurgitation............................e28 3.5.1. PathophysiologyandNaturalHistory............e55 3.2.2.1. Pathophysiology................................e28 3.5.2. EvaluationandManagementofthe 3.2.2.2. Diagnosis...........................................e28 AsymptomaticPatient.....................................e56 3.2.2.3. Treatment..........................................e28 3.5.3. EvaluationandManagementofthe 3.2.3. ChronicAorticRegurgitation.........................e28 SymptomaticPatient.......................................e57 Downloaded from content.onlinejacc.org by on September 1, 2006 JACCVol.48,No.3,2006 Bonowetal. e3 August1,2006:e1–148 ACC/AHAPracticeGuidelines 3.5.4. SurgicalConsiderations...................................e59 3.7.5. CombinedMitralRegurgitationandAortic 3.6. MitralRegurgitation..................................................e59 Regurgitation...................................................e69 3.6.1. Etiology...........................................................e59 3.7.5.1. Pathophysiology................................e69 3.6.2. AcuteSevereMitralRegurgitation.................e59 3.7.5.2. DiagnosisandTherapy.....................e70 3.6.2.1. Pathophysiology................................e59 3.7.6. CombinedMitralStenosisandAortic 3.6.2.2. Diagnosis...........................................e59 Stenosis............................................................e70 3.6.2.3. MedicalTherapy...............................e59 3.7.6.1. Pathophysiology................................e70 3.6.3. ChronicAsymptomaticMitral 3.7.6.2. DiagnosisandTherapy.....................e70 Regurgitation...................................................e60 3.7.7. CombinedAorticStenosisandMitral 3.6.3.1. PathophysiologyandNatural Regurgitation...................................................e70 History..............................................e60 3.7.7.1. Pathophysiology................................e70 3.6.3.2. Diagnosis...........................................e60 3.7.7.2. DiagnosisandTherapy.....................e70 3.6.3.3. IndicationsforTransthoracic 3.8. TricuspidValveDisease.............................................e70 Echocardiography..............................e60 3.8.1. Pathophysiology..............................................e70 3.6.3.4. IndicationsforTransesophageal 3.8.2. Diagnosis.........................................................e71 Echocardiography..............................e61 3.8.3. Management...................................................e71 3.6.3.5. SerialTesting....................................e61 3.9. Drug-RelatedValvularHeartDisease.......................e72 3.6.3.6. GuidelinesforPhysicalActivity 3.10. RadiationHeartDisease............................................e72 andExercise......................................e62 3.6.3.7. MedicalTherapy...............................e62 4. EVALUATIONANDMANAGEMENTOF 3.6.3.8. IndicationsforCardiac INFECTIVEENDOCARDITIS....................................e73 Catheterization..................................e62 4.1. AntimicrobialTherapy...............................................e73 3.6.4. IndicationsforSurgery....................................e63 4.2. Culture-NegativeEndocarditis..................................e74 3.6.4.1. TypesofSurgery...............................e63 4.3. EndocarditisinHIV-SeropositivePatients...............e76 3.6.4.2. IndicationsforMitralValve 4.4. IndicationsforEchocardiographyinSuspectedor Operation..........................................e63 KnownEndocarditis..................................................e76 3.6.4.2.1. SymptomaticPatients 4.4.1. TransthoracicEchocardiographyin WithNormalLeft Endocarditis....................................................e77 VentricularFunction........e64 4.4.2. TransesophagealEchocardiographyin 3.6.4.2.2. Asymptomaticor Endocarditis....................................................e78 SymptomaticPatients 4.5. OutpatientTreatment................................................e80 WithLeftVentricular 4.6. IndicationsforSurgeryinPatientsWithAcute Dysfunction......................e64 InfectiveEndocarditis................................................e80 3.6.4.2.3. AsymptomaticPatients 4.6.1. SurgeryforNativeValveEndocarditis...........e82 WithNormalLeft 4.6.2. SurgeryforProstheticValveEndocarditis.....e82 VentricularFunction........e66 3.6.4.2.4. AtrialFibrillation.............e66 5. MANAGEMENTOFVALVULARDISEASEIN 3.6.5. IschemicMitralRegurgitation........................e67 PREGNANCY..................................................................e83 3.6.6. EvaluationofPatientsAfterMitralValve 5.1. PhysiologicalChangesofPregnancy.........................e83 ReplacementorRepair....................................e67 5.2. PhysicialExamination................................................e83 3.6.7. SpecialConsiderationsintheElderly............e67 5.3. Echocardiography.......................................................e83 3.7. MultipleValveDisease..............................................e67 5.4. GeneralManagementGuidelines..............................e84 3.7.1. Introduction....................................................e67 5.5. SpecificLesions..........................................................e84 3.7.2. MixedSingleValveDisease...........................e68 5.5.1. MitralStenosis................................................e84 3.7.2.1. Pathophysiology................................e68 5.5.2. MitralRegurgitation.......................................e86 3.7.2.2. Diagnosis...........................................e68 5.5.3. AorticStenosis................................................e86 3.7.2.2.1. Two-Dimensionaland 5.5.4. AorticRegurgitation.......................................e87 DopplerEchocardiographic 5.5.5. PulmonicStenosis...........................................e87 Studies..............................e68 5.5.6. TricuspidValveDisease..................................e87 3.7.2.2.2. CardiacCatheterization...e68 5.5.7. MarfanSyndrome...........................................e87 3.7.2.3. Management.....................................e69 5.6. EndocarditisProphylaxis............................................e87 3.7.3. CombinedMitralStenosisandAortic 5.7. CardiacValveSurgery................................................e88 Regurgitation...................................................e69 5.8. AnticoagulationDuringPregnancy...........................e88 3.7.3.1. Pathophysiology................................e69 5.8.1. Warfarin..........................................................e88 3.7.3.2. Management.....................................e69 5.8.2. UnfractionatedHeparin..................................e88 3.7.4. CombinedMitralStenosisandTricuspid 5.8.3. Low-Molecular-WeightHeparins..................e88 Regurgitation...................................................e69 5.8.4. SelectionofAnticoagulationRegimenin 3.7.4.1. Pathophysiology................................e69 PregnantPatientsWithMechanical 3.7.4.2. Diagnosis...........................................e69 ProstheticValves.............................................e89 3.7.4.3. Management.....................................e69 5.9. SelectionofValveProsthesesinYoungWomen......e90 Downloaded from content.onlinejacc.org by on September 1, 2006 e4 Bonowetal. JACCVol.48,No.3,2006 ACC/AHAPracticeGuidelines August1,2006:e1–148 6. MANAGEMENTOFCONGENITALVALVULAR 8.1.1. AorticStenosis..............................................e110 HEARTDISEASEINADOLESCENTSAND 8.1.2. AorticRegurgitation.....................................e110 YOUNGADULTS...........................................................e90 8.1.3. MitralStenosis..............................................e110 6.1. AorticStenosis...........................................................e91 8.1.4. MitralRegurgitation.....................................e111 6.1.1. Pathophysiology..............................................e91 8.1.5. TricuspidRegurgitation................................e111 6.1.2. EvaluationofAsymptomaticAdolescentsor 8.1.6. TricuspidStenosis.........................................e111 YoungAdultsWithAorticStenosis..............e91 8.1.7. PulmonicValveLesions................................e111 6.1.3. IndicationsforAorticBalloonValvotomyin 8.2. SpecificClinicalScenarios.......................................e111 AdolescentsandYoungAdults.......................e92 8.2.1. PreviouslyUndetectedAorticStenosisDuring 6.2. AorticRegurgitation..................................................e93 CABG...........................................................e111 6.3. MitralRegurgitation..................................................e94 8.2.2. PreviouslyUndetectedMitralRegurgitation 6.4. MitralStenosis...........................................................e95 DuringCABG..............................................e111 6.5. TricuspidValveDisease.............................................e95 9. MANAGEMENTOFPATIENTSWITH 6.5.1. Pathophysiology..............................................e95 PROSTHETICHEARTVALVES..............................e112 6.5.2. EvaluationofTricuspidValveDiseasein 9.1. AntibioticProphylaxis..............................................e112 AdolescentsandYoungAdults.......................e96 9.1.1. InfectiveEndocarditis...................................e112 6.5.3. IndicationsforInterventioninTricuspid 9.1.2. RecurrenceofRheumaticCarditis................e112 Regurgitation...................................................e96 9.2. AntithromboticTherapy..........................................e112 6.6. PulmonicStenosis......................................................e97 9.2.1. MechanicalValves.........................................e113 6.6.1. Pathophysiology..............................................e97 9.2.2. BiologicalValves...........................................e114 6.6.2. EvaluationofPulmonicStenosisin 9.2.3. EmbolicEventsDuringAdequate AdolescentsandYoungAdults.......................e97 AntithromboticTherapy...............................e114 6.6.3. IndicationsforBalloonValvotomyin 9.2.4. ExcessiveAnticoagulation.............................e114 PulmonicStenosis...........................................e97 9.2.5. BridgingTherapyinPatientsWithMechanical 6.7. PulmonaryRegurgitation...........................................e98 ValvesWhoRequireInterruptionofWarfarin 7. SURGICALCONSIDERATIONS................................e99 TherapyforNoncardiacSurgery,Invasive 7.1. AmericanAssociationforThoracicSurgery/ Procedures,orDentalCare..........................e114 SocietyofThoracicSurgeonsGuidelinesforClinical 9.2.6. AntithromboticTherapyinPatientsWhoNeed ReportingofHeartValveComplications..................e99 CardiacCatheterization/Angiography..........e115 7.2. AorticValveSurgery................................................e101 9.2.7. ThrombosisofProstheticHeartValves.......e115 7.2.1. RisksandStrategiesinAorticValveSurgery..e101 9.3. Follow-UpVisits......................................................e116 7.2.2. MechanicalAorticValveProstheses.............e101 9.3.1. FirstOutpatientPostoperativeVisit.............e117 7.2.2.1. AntithromboticTherapyforPatients 9.3.2. Follow-UpVisitsinPatientsWithout WithAorticMechanicalHeartValves.e101 Complications...............................................e117 7.2.3. StentedandNonstentedHeterografts..........e102 9.3.3. Follow-UpVisitsinPatientsWith 7.2.3.1. AorticValveReplacementWith Complications...............................................e117 StentedHeterografts.......................e102 9.4. ReoperationtoReplaceaProstheticValve.............e118 7.2.3.2. AorticValveReplacementWith 10. EVALUATIONANDTREATMENTOFCORONARY StentlessHeterografts.....................e102 ARTERYDISEASEINPATIENTSWITH 7.2.4. AorticValveHomografts..............................e102 VALVULARHEARTDISEASE.................................e118 7.2.5. PulmonicValveAutotransplantation............e104 10.1. ProbabilityofCoronaryArteryDiseaseinPatients 7.2.6. AorticValveRepair......................................e104 WithValvularHeartDisease...................................e118 7.2.7. LeftVentricle–to–DescendingAortaShunt.e104 10.2. DiagnosisofCoronaryArteryDisease....................e119 7.2.8. ComparativeTrialsandSelectionofAortic 10.3. TreatmentofCoronaryArteryDiseaseattheTimeof ValveProstheses............................................e104 AorticValveReplacement.......................................e120 7.2.9. MajorCriteriaforAorticValveSelection....e105 10.4. AorticValveReplacementinPatientsUndergoing 7.3. MitralValveSurgery................................................e106 CoronaryArteryBypassSurgery..............................e120 7.3.1. MitralValveRepair......................................e106 10.5. ManagementofConcomitantMitralValveDisease 7.3.1.1. MyxomatousMitralValve..............e106 andCoronaryArteryDisease...................................e121 7.3.1.2. RheumaticHeartDisease...............e107 APPENDIX1.........................................................................e121 7.3.1.3. IschemicMitralValveDisease.......e107 APPENDIX2.........................................................................e122 7.3.1.4. MitralValveEndocarditis...............e107 APPENDIX3.........................................................................e124 7.3.2. MitralValveProstheses(Mechanicalor REFERENCES......................................................................e124 Bioprostheses)...............................................e107 7.3.2.1. SelectionofaMitralValve PREAMBLE Prosthesis.........................................e108 7.4. TricuspidValveSurgery...........................................e108 Itisimportantthatthemedicalprofessionplayasignificant 7.5. ValveSelectionforWomenofChildbearingAge......e109 role in critically evaluating the use of diagnostic procedures 8. INTRAOPERATIVEASSESSMENT.........................e109 and therapies as they are introduced in the detection, 8.1. SpecificValveLesions..............................................e110 management, or prevention of disease states. Rigorous and Downloaded from content.onlinejacc.org by on September 1, 2006 JACCVol.48,No.3,2006 Bonowetal. e5 August1,2006:e1–148 ACC/AHAPracticeGuidelines expert analysis of the available data documenting the abso- andhttp://circ.ahajournals.org/manual).SeeAppendix1fora lute and relative benefits and risks of those procedures and list of writing committee member relationships with industry therapies can produce helpful guidelines that improve the andAppendix2foralistingofpeerreviewerrelationshipswith effectiveness of care, optimize patient outcomes, and favor- industrythatarepertinenttothisguideline. ably affect the overall cost of care by focusing resources on Thesepracticeguidelinesareintendedtoassisthealthcare the most effective strategies. providers in clinical decision making by describing a range The American College of Cardiology (ACC) and the of generally acceptable approaches for the diagnosis, man- American Heart Association (AHA) have jointly engaged agement, and prevention of specific diseases or conditions. in the production of such guidelines in the area of cardio- These guidelines attempt to define practices that meet the vascular disease since 1980. This effort is directed by the needsofmostpatientsinmostcircumstances.Theseguide- ACC/AHA Task Force on Practice Guidelines, whose line recommendations reflect a consensus of expert opinion chargeistodevelop,update,orrevisepracticeguidelinesfor after a thorough review of the available, current scientific important cardiovascular diseases and procedures. Writing evidence and are intended to improve patient care. If these committees are charged with the task of performing an guidelines are used as the basis for regulatory/payer deci- assessment of the evidence and acting as an independent sions, the ultimate goal is quality of care and serving the groupofauthorstodevelopandupdatewrittenrecommen- patient’s best interests. The ultimate judgment regarding dations for clinical practice. care of a particular patient must be made by the healthcare Experts in the subject under consideration are selected provider and patient in light of all of the circumstances from both organizations to examine subject-specific data presentedbythatpatient.Therearecircumstancesinwhich and write guidelines. The process includes additional rep- deviations from these guidelines are appropriate. resentatives from other medical practitioner and specialty The “ACC/AHA 2006 Guideline for the Management groups where appropriate. Writing committees are specifi- ofPatientsWithValvularHeartDisease”wasapprovedfor cally charged to perform a formal literature review, weigh publication by the ACC Foundation (ACCF) board of thestrengthofevidencefororagainstaparticulartreatment trustees in May 2006 and the AHA Science Advisory and or procedure, and include estimates of expected health Coordinating Committee in May 2006. The executive outcomes where data exist. Patient-specific modifiers, co- summaryandrecommendationsarepublishedintheAugust morbidities, and issues of patient preference that might 1, 2006 issue of the Journal of the American College of influence the choice of particular tests or therapies are CardiologyandtheAugust1,2006issueofCirculation.The considered, as well as frequency of follow-up. When avail- full-text guideline is e-published in the same issues of each able, information from studies on cost will be considered; journal and is posted on the World Wide Web sites of the however, review of data on efficacy and clinical outcomes ACC (www.acc.org) and the AHA (www.american- will be the primary basis for preparing recommendation in heart.org). The guidelines will be reviewed annually by the these guidelines. ACC/AHATaskForceonPracticeGuidelinesandwillbe The ACC/AHA Task Force on Practice Guidelines considered current unless they are updated, revised, or makes every effort to avoid any actual, potential, or per- sunsetted and withdrawn from distribution. Copies of the ceived conflicts of interest that might arise as a result of an fulltextandtheexecutivesummaryareavailablefromboth outsiderelationshiporpersonalinterestofamemberofthe organizations. writing committee. Specifically, all members of the writing Sidney C. Smith, Jr., MD, FACC, FAHA, committeeandpeerreviewersofthedocumentareaskedto Chair, ACC/AHA Task Force on Practice Guidelines provide disclosure statements of all such relationships that might be perceived as real or potential conflicts of interest. 1. INTRODUCTION Writing committee members are also strongly encouraged to declare a previous relationship with industry that might 1.1. Organization of the Committee and Evidence Review be perceived as relevant to guideline development. If a writing committee member develops a new relationship The ACC and the AHA have long been involved in the withindustryduringhisorhertenure,heorsheisrequired joint development of practice guidelines designed to assist to notify guideline staff in writing. The continued partici- healthcare providers in the management of selected cardio- pation of the writing committee member will be reviewed. vascular disorders or the selection of certain cardiovascular These statements are reviewed by the parent task force, procedures. The determination of the disorders or proce- reported orally to all members of the writing panel at each dures to develop guidelines for is based on several factors, meeting,andupdatedandreviewedbythewritingcommit- including importance to healthcare providers and whether tee as changes occur. Please refer to the methodology there are sufficient data from which to derive accepted manual for the ACC/AHA guideline writing committees guidelines.Oneimportantcategoryofcardiacdisordersthat for further description and the relationships with industry affect a large number of patients who require diagnostic policy,availableonACCandAHAWorldWideWebsites proceduresanddecisionsregardinglong-termmanagement (http://www.acc.org/clinical/manual/manual_introltr.htm is valvular heart disease. Downloaded from content.onlinejacc.org by on September 1, 2006 e6 Bonowetal. JACCVol.48,No.3,2006 ACC/AHAPracticeGuidelines August1,2006:e1–148 Duringthepast2decades,majoradvanceshaveoccurredin Classification of recommendations and level of evidence diagnostic techniques, the understanding of natural history, are expressed in the ACC/AHA format as follows: and interventional cardiology and surgical procedures for pa- • Class I: Conditions for which there is evidence for tientswithvalvularheartdisease.Theseadvanceshaveresulted and/or general agreement that the procedure or treat- inenhanceddiagnosis,morescientificselectionofpatientsfor ment is beneficial, useful, and effective. surgeryorcatheter-basedinterventionversusmedicalmanage- • Class II: Conditions for which there is conflicting ment, and increased survival of patients with these disorders. evidence and/or a divergence of opinion about the The information base from which to make clinical manage- usefulness/efficacy of a procedure or treatment. ment decisions has greatly expanded in recent years, yet in • Class IIa: Weight of evidence/opinion is in favor of many situations, management issues remain controversial or usefulness/efficacy. uncertain.Unlikemanyotherformsofcardiovasculardisease, • Class IIb: Usefulness/efficacy is less well established thereisascarcityoflarge-scalemulticentertrialsaddressingthe by evidence/opinion. diagnosisandtreatmentofpatientswithvalvulardiseasefrom • ClassIII:Conditionsforwhichthereisevidenceand/or which to derive definitive conclusions, and the information general agreement that the procedure/treatment is not available in the literature represents primarily the experiences useful/effective and in some cases may be harmful. reported by single institutions in relatively small numbers of patients. In addition, the weight of evidence in support of the The 1998 Committee on Management of Patients With recommendation is listed as follows: Valvular Heart Disease reviewed and compiled this informa- tion base and made recommendations for diagnostic testing, • Level of Evidence A: Data derived from multiple treatment,andphysicalactivity.Fortopicsforwhichtherewas randomized clinical trials. an absence of multiple randomized, controlled trials, the • Level of Evidence B: Data derived from a single preferredbasisformedicaldecisionmakinginclinicalpractice randomized trial or nonrandomized studies. (evidence-basedmedicine),thecommittee’srecommendations • Level of Evidence C: Only consensus opinion of ex- werebasedondataderivedfromsinglerandomizedtrialsor perts, case studies, or standard-of-care. nonrandomized studies or were based on a consensus The schema for classification of recommendations and opinion of experts. The current writing committee was level of evidence is summarized in Figure 1, which also chargedwithrevisingtheguidelinespublishedin1998.The illustrates how the grading system provides an estimate of committee reviewed pertinent publications, including ab- the size of the treatment effect and an estimate of the stracts, through a computerized search of the English certainty of the treatment effect. literaturesince1998andperformedamanualsearchoffinal Writing committee membership consisted of cardiovas- articles. Special attention was devoted to identification of cular disease specialists and representatives of the cardiac randomizedtrialspublishedsincetheoriginaldocument.A surgeryandcardiacanesthesiologyfields;boththeacademic completelistingofallpublicationscoveringthetreatmentof andprivatepracticesectorswererepresented.TheSocietyof valvularheartdiseaseisbeyondthescopeofthisdocument; Cardiovascular Anesthesiologists assigned an official repre- the document includes those reports that the committee sentative to the writing committee. believes represent the most comprehensive or convincing This document was reviewed by 2 official reviewers datathatarenecessarytosupportitsconclusions.However, nominated by the ACC; 2 official reviewers nominated by evidencetableswereupdatedtoreflectmajoradvancesover the AHA; 1 official reviewer from the ACC/AHA Task this time period. Inaccuracies or inconsistencies present in Force on Practice Guidelines; reviewers nominated by the theoriginalpublicationwereidentifiedandcorrectedwhen SocietyofCardiovascularAnesthesiologists,theSocietyfor possible. Recommendations provided in this document are Cardiovascular Angiography and Interventions, and the based primarily on published data. Because randomized SocietyofThoracicSurgeons(STS);andindividualcontent trialsareunavailableinmanyfacetsofvalvularheartdisease reviewers, including members of the ACCF Cardiac Cath- treatment, observational studies and, in some areas, expert eterizationandInterventionCommittee,ACCFCardiovas- opinionsformthebasisforrecommendationsthatareoffered. cular Imaging Committee, ACCF Cardiovascular Surgery All of the recommendations in this guideline revision Committee,AHAEndocarditisCommittee,AHACardiac were converted from the tabular format used in the 1998 Clinical Imaging Committee, AHA Cardiovascular Inter- guideline to a listing of recommendations that has been ventionandImagingCommittee,andAHACerebrovascu- writteninfullsentencestoexpressacompletethought,such lar Imaging and Intervention Committee. that a recommendation, even if separated and presented apartfromtherestofthedocument,wouldstillconveythe 1.2. Scope of the Document fullintentoftherecommendation.Itishopedthatthiswill increasethereaders’comprehensionoftheguidelines.Also, The guidelines attempt to deal with general issues of the level of evidence, either A, B, or C, for each recom- treatment of patients with heart valve disorders, such as mendation is now provided. evaluation of patients with heart murmurs, prevention and Downloaded from content.onlinejacc.org by on September 1, 2006 AugJAC uC s tV 1,ol. 24 08 0, 6N :e1o. –3 1, 4820 0 6 D o w n lo a d e d fro m c o n te n t.o n lin e ja c c .o rg b y o n S e p te m b e r 1 , 2 0 A 0 C 6 C/A H A P r a c ticeB o Figure1. Applyingclassificationofrecommendationsandlevelofevidence.*Dataavailablefromclinicaltrialsorregistriesabouttheusefulness/efficacyindifferentsubpopulations,suchasgender, Gn uo age,historyofdiabetes,historyofpriormyocardialinfarction,historyofheartfailure,andprioraspirinuse.ArecommendationwithLevelofEvidenceBorCdoesnotimplythattherecommendation idw isweak.Manyimportantclinicalquestionsaddressedintheguidelinesdonotlendthemselvestoclinicaltrials.Eventhoughrandomizedtrialsarenotavailable,theremaybeaveryclearclinical elinet consensus that a particular test or therapy is useful or effective. †In 2003 the ACC/AHA Task Force on Practice Guidelines recently provided a list of suggested phrases to use when writing ea sl. recommendations.Allrecommendationsinthisguidelinehavebeenwritteninfullsentencesthatexpressacompletethought,suchthatarecommendation,evenifseparatedandpresentedapart fromtherestofthedocument(includingheadingsabovesetsofrecommendations),wouldstillconveythefullintentoftherecommendation.Itishopedthatthiswillincreasereaders’comprehension oftheguidelinesandwillallowqueriesattheindividualrecommendationlevel. e 7 e8 Bonowetal. JACCVol.48,No.3,2006 ACC/AHAPracticeGuidelines August1,2006:e1–148 treatment of endocarditis, management of valve disease in Often, more than 1 of these factors is operative (5–7). pregnancy, and treatment of patients with concomitant Aheartmurmurmayhavenopathologicalsignificanceor coronary artery disease (CAD), as well as more specialized may be an important clue to the presence of valvular, issues that pertain to specific valve lesions. The guidelines congenital,orotherstructuralabnormalitiesoftheheart(8). focusprimarilyonvalvularheartdiseaseintheadult,witha Mostsystolicheartmurmursdonotsignifycardiacdisease, separate section dealing with specific recommendations for andmanyarerelatedtophysiologicalincreasesinbloodflow valve disorders in adolescents and young adults. The diag- velocity (9). In other instances, a heart murmur may be an nosis and management of infants and young children with importantcluetothediagnosisofundetectedcardiacdisease congenital valvular abnormalities are significantly different (e.g., valvular aortic stenosis [AS]) that may be important from those of the adolescent or adult and are beyond the even when asymptomatic or that may define the reason for scope of these guidelines. cardiac symptoms. In these situations, various noninvasive This task force report overlaps with several previously orinvasivecardiactestsmaybenecessarytoestablishafirm publishedACC/AHAguidelinesaboutcardiacimagingand diagnosis and form the basis for rational treatment of an diagnostic testing, including the guidelines for the clinical underlyingdisorder.Echocardiographyisparticularlyuseful use of cardiac radionuclide imaging (1), the clinical appli- in this regard, as discussed in the “ACC/AHA/ASE 2003 cation of echocardiography (2), exercise testing (3), and Guidelines for the Clinical Application of Echocardiogra- percutaneous coronary intervention (4). Although these phy” (2). Diastolic murmurs virtually always represent guidelines are not intended to include detailed information pathological conditions and require further cardiac evalua- covered in previous guidelines on the use of imaging and tion, as do most continuous murmurs. Continuous “inno- diagnostic testing, an essential component of this report is cent”murmursincludevenoushumsandmammarysouffles. thediscussionofindicationsforthesetestsintheevaluation The traditional auscultation method of assessing cardiac and treatment of patients with valvular heart disease. murmurshasbeenbasedontheirtiminginthecardiaccycle, The committee emphasizes the fact that many factors configuration, location and radiation, pitch, intensity ultimately determine the most appropriate treatment of (grades1through6),andduration(5–9).Theconfiguration individualpatientswithvalvularheartdiseasewithinagiven of a murmur may be crescendo, decrescendo, crescendo- community. These include the availability of diagnostic decrescendo (diamond-shaped), or plateau. The precise equipmentandexpertdiagnosticians,theexpertiseofinter- times of onset and cessation of a murmur associated with ventional cardiologists and surgeons, and notably, the cardiac pathology depend on the period of time in the wishesofwell-informedpatients.Therefore,deviationfrom cardiac cycle in which a physiologically important pressure theseguidelinesmaybeappropriateinsomecircumstances. differencebetween2chambersoccurs(5–9).Aclassification These guidelines are written with the assumption that a of cardiac murmurs is listed in Table 1. diagnostic test can be performed and interpreted with skill 2.1.2. Classification of Murmurs levelsconsistentwithpreviouslyreportedACCtrainingand competency statements and ACC/AHA guidelines, that Holosystolic (pansystolic) murmurs are generated when there interventional cardiological and surgical procedures can be isflowbetweenchambersthathavewidelydifferentpressures performedbyhighlytrainedpractitionerswithinacceptable throughoutsystole,suchastheleftventricleandeithertheleft safetystandards,andthattheresourcesnecessarytoperform atriumorrightventricle.Withanabnormalregurgitantorifice, thesediagnosticproceduresandprovidethiscarearereadily the pressure gradient and regurgitant jet begin early in con- available. This is not true in all geographic areas, which tractionandlastuntilrelaxationisalmostcomplete. furtherunderscoresthecommittee’spositionthatitsrecom- Midsystolic (systolic ejection) murmurs, often crescendo- mendations are guidelines and not rigid requirements. decrescendo in configuration, occur when blood is ejected acrosstheaorticorpulmonicoutflowtracts.Themurmursstart 2. GENERAL PRINCIPLES shortlyafterS ,whentheventricularpressurerisessufficiently 1 2.1. Evaluation of the Patient With a Cardiac Murmur Table1. ClassificationofCardiacMurmurs 2.1.1. Introduction 1.Systolicmurmurs a.Holosystolic(pansystolic)murmurs Cardiac auscultation remains the most widely used method b.Midsystolic(systolicejection)murmurs of screening for valvular heart disease. The production of c.Earlysystolicmurmurs murmurs is due to 3 main factors: d.Midtolatesystolicmurmurs • highbloodflowratethroughnormalorabnormalorifices 2.Diastolicmurmurs a.Earlyhigh-pitcheddiastolicmurmurs • forwardflowthroughanarrowedorirregularorificeinto b.Middiastolicmurmurs a dilated vessel or chamber c.Presystolicmurmurs • backward or regurgitant flow through an incompetent 3.Continuousmurmurs valve Downloaded from content.onlinejacc.org by on September 1, 2006
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