PRACTICE GUIDELINE 2014 AHA/ACC Guideline for the

51 Pages·2014·3.12 MB·English

JournaloftheAmericanCollegeofCardiology Vol.63,No.22,2014
(cid:1)2014bytheAmericanHeartAssociation,Inc.,andtheAmericanCollegeofCardiologyFoundation ISSN0735-1097/$36.00
PublishedbyElsevierInc. http://dx.doi.org/10.1016/j.jacc.2014.02.537
PRACTICE GUIDELINE
2014 AHA/ACC Guideline for the Management of
Patients With Valvular Heart Disease: Executive Summary
A Report of the American College of Cardiology/American Heart Association
Task Force on Practice Guidelines
Developed in Collaboration With the American Association for Thoracic Surgery,
American Society of Echocardiography, Society for Cardiovascular Angiography and Interventions,
Society of Cardiovascular Anesthesiologists, and Society of Thoracic Surgeons
Writing Rick A. Nishimura, MD, MACC, FAHA, Paul Sorajja, MD, FACC, FAHA#
Committee Co-Chairy Thoralf M. Sundt III, MD* **yy
Members* Catherine M. Otto, MD, FACC, FAHA, JamesD.Thomas,MD,FASE,FACC,FAHAzz
Co-Chairy
*Writing committee members are required to recuse themselves from
voting on sections to which their specific relationships with industry
Robert O. Bonow, MD, MACC, FAHAy andother entities mayapply; see Appendix 1 forrecusal information.
Blase A. Carabello, MD, FACC*y yACC/AHArepresentative.zACC/AHATaskForceonPerformance
John P. Erwin III, MD, FACC, FAHAz Mliaiesaosnu.res{Sloiaciiseotny. oxfACCCar/dAioHvaAscuTlaarskAFnoesrtcheesoionlogPirsatscticreeprGesueindtealtiinvees.
Robert A. Guyton, MD, FACC*x #SocietyforCardiovascularAngiographyandInterventionsrepresenta-
Patrick T. O’Gara, MD, FACC, FAHAy tive. **American Association for Thoracic Surgery representative.
yySociety of Thoracic Surgeons representative. zzAmerican Society of
Carlos E. Ruiz, MD, PHD, FACCy Echocardiographyrepresentative.
Nikolaos J. Skubas, MD, FASE{
ACC/AHATask Jeffrey L. Anderson, MD, FACC, FAHA, Robert A. Guyton, MD, FACCxx
ForceMembers Chair Judith S. Hochman, MD, FACC, FAHA
Jonathan L. Halperin, MD, FACC, FAHA, Richard J. Kovacs, MD, FACC, FAHA
Chair-Elect E. Magnus Ohman, MD, FACC
Susan J. Pressler, PHD, RN, FAHA
NancyM.Albert,PHD,CCNS,CCRN,FAHA Frank W. Sellke, MD, FACC, FAHA
Biykem Bozkurt, MD, PHD, FACC, FAHA Win-Kuang Shen, MD, FACC, FAHA
Ralph G. Brindis, MD, MPH, MACC William G. Stevenson, MD, FACC, FAHAxx
Mark A. Creager, MD, FACC, FAHAxx Clyde W. Yancy, MD, FACC, FAHAxx
Lesley H. Curtis, PHD, FAHA
David DeMets, PHD xxTaskForcememberduringthewritingeffort.
Full-textguidelineavailableat:JAmCollCardiol2014;xx:xxx-xxx. ThisarticlehasbeencopublishedinCirculation.
This document was approved by the American College of Cardiology Board Copies: This document is available on the World Wide Web sites of the
of Trustees and the American Heart Association Science Advisory and Coordi- AmericanCollegeofCardiology(www.cardiosource.org)andtheAmericanHeart
natingCommitteeinJanuary2014. Association(my.americanheart.org).Forcopiesofthisdocument,pleasecontact
The American College of Cardiology requests that this document theElsevierInc.ReprintDepartmentviafax(212)462-1935ore-mailreprints@
be cited as follows: Nishimura RA, Otto CM, Bonow RO, Carabello BA, elsevier.com.
Erwin JP III, Guyton RA, O’Gara PT, Ruiz CE, Skubas NJ, Sorajja P, Permissions:Multiplecopies,modification,alteration,enhancement,and/or
Sundt TM III, Thomas JD. 2014 AHA/ACC guideline for the manage- distributionofthisdocumentarenotpermittedwithouttheexpresspermission
mentofpatientswithvalvularheartdisease:executivesummary:areportof oftheAmericanCollegeofCardiology.Requestsmaybecompletedonlinevia
the American College of Cardiology/American Heart Association Task the Elsevier site (http://www.elsevier.com/authors/obtaining-permission-to-
Force on Practice Guidelines. J Am Coll Cardiol 2014;63:xxx–xxx. re-use-elsevier-material).
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7.3. ChronicSecondaryMR ..................... 23
TABLEOFCONTENTS
7.3.1. DiagnosisandFollow-Up ............... 23
7.3.2. MedicalTherapy....................... 24
Preamble.............................................3
7.3.3. Intervention ........................... 24
8. TricuspidValveDisease:
1. Introduction .....................................5 Recommendations..............................24
1.1. MethodologyandEvidenceReview ...........5 8.1. StagesofTR ............................... 24
1.2. OrganizationoftheWritingCommittee .......5 8.2. TricuspidRegurgitation..................... 25
1.3. DocumentReviewandApproval ..............5
8.2.1. DiagnosisandFollow-Up ............... 25
1.4. ScopeoftheGuideline .......................6 8.2.2. MedicalTherapy....................... 25
8.2.3. Intervention ........................... 25
2. General Principles ...............................6
8.3. StagesofTricuspidStenosis ............... 26
8.4. TricuspidStenosis ......................... 26
2.1. EvaluationofthePatientWith
SuspectedVHD ..............................6 8.4.1. DiagnosisandFollow-Up ............... 26
2.2. DefinitionsofSeverityofValveDisease ......7 8.4.2. Intervention ........................... 26
2.3. DiagnosticTestingdDiagnosisandFollow-Up: 9. StagesofPulmonic ValveDisease .............26
Recommendations ...........................7
2.4. BasicPrinciplesofMedicalTherapy: 10. Prosthetic Valves:Recommendations.........26
Recommendations ...........................8
2.5. EvaluationofSurgicalandInterventionalRisk .8 10.1. EvaluationandSelectionof
2.6. TheHeartValveTeamandHeartValveCenters ProstheticValves ........................26
ofExcellence:Recommendations.............9 10.1.1. DiagnosisandFollow-Up ............26
10.1.2. Intervention ........................26
3. AorticStenosis:Recommendations ............11
10.2. AntithromboticTherapyfor
ProstheticValves ........................26
3.1. StagesofValvularAS ...................... 12
10.3. BridgingTherapyforProsthetic
3.2. DiagnosisandFollow-Up.................... 14 Valves...................................27
3.3. MedicalTherapy............................ 15 10.4. ExcessiveAnticoagulationand
3.4. TimingofIntervention ...................... 15 SeriousBleedingWithProsthetic
3.5. ChoiceofIntervention ..................... 17 Valves...................................27
10.5. ProstheticValveThrombosis .............27
4. AorticRegurgitation: Recommendations .......18
10.5.1. DiagnosisandFollow-Up ............27
10.5.2. MedicalTherapy....................28
4.1. StagesofChronicAorticRegurgitation ..... 18
10.5.3. Intervention ........................28
4.2. DiagnosisandFollow-Up.................... 19
10.6. ProstheticValveStenosis................28
4.3. MedicalTherapy............................ 19
10.7. ProstheticValveRegurgitation ...........28
4.4. TimingofIntervention ...................... 19
11. InfectiveEndocarditis:
5. BicuspidAorticValve andAortopathy:
Recommendations ............................28
Recommendations..............................19
11.1. DiagnosisandFollow-Up .................28
5.1. DiagnosisandFollow-Up.................... 19
11.2. MedicalTherapy .........................30
5.2. Intervention................................ 19
11.3. Intervention .............................30
6. MitralStenosis:Recommendations ............20
12. PregnancyandVHD:Recommendations.......31
6.1. StagesofMS .............................. 20
6.2. DiagnosisandFollow-Up.................... 21 12.1. NativeValveStenosis....................31
6.3. MedicalTherapy............................ 21 12.1.1. DiagnosisandFollow-Up ............32
12.1.2. MedicalTherapy....................32
6.4. Intervention................................ 21
12.1.3. Intervention ........................32
7. MitralRegurgitation: Recommendations .......22 12.2. NativeValveRegurgitation...............32
12.2.1. DiagnosisandFollow-Up ............32
7.1. StagesofChronicMR ...................... 22 12.2.2. MedicalTherapy....................32
7.2. ChronicPrimaryMR ........................ 22 12.2.3. Intervention ........................32
12.3. ProstheticValvesinPregnancy ..........33
7.2.1. DiagnosisandFollow-Up ............... 22
7.2.2. MedicalTherapy....................... 23 12.3.1. DiagnosisandFollow-Up ............33
7.2.3. Intervention ........................... 23 12.3.2. MedicalTherapy....................33
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13. SurgicalConsiderations: Recommendations ..33 cost effectiveness. When available, information from
studiesoncostisconsidered; however,areviewof data on
13.1. EvaluationofCoronaryAnatomy .........33 efficacy and outcomes constitutes the primary basis for
13.2. ConcomitantProcedures .................34 preparing recommendations in this guideline.
13.2.1. InterventionforCAD ...............34 In analyzing the data and developing recommendations
13.2.2. InterventionforAF .................34 and supporting text, the writing committee uses evidence-
basedmethodologiesdevelopedbytheTaskForce(1).The
14. NoncardiacSurgeryinPatientsWith VHD:
Recommendations ............................35 ClassofRecommendation(COR)isanestimateofthesize
of the treatment effect, with consideration given to risks
References..........................................36 versusbenefits,aswellasevidenceand/oragreementthata
giventreatmentorprocedureisorisnotuseful/effectiveor
Appendix 1. Author Relationships WithIndustry insomesituationsmaycauseharm.TheLevelofEvidence
andOtherEntities(Relevant) .......................46 (LOE) is an estimate of the certainty or precision of the
treatmenteffect.Thewritingcommitteereviewsandranks
Appendix 2. Reviewer Relationships WithIndustry evidence supporting each recommendation, with the
andOtherEntities(Relevant) .......................48 weight of evidence ranked as LOE A, B, or C, according
tospecificdefinitions.TheschemafortheCORandLOE
is summarized in Table 1, which also provides suggested
phrases for writing recommendations within each COR.
Studies are identified as observational, retrospective,
prospective, or randomized, as appropriate. For certain
Preamble
conditions for which inadequate data are available, rec-
ommendations are based on expert consensus and clinical
The medical profession should play a central role in experience and are ranked as LOE C. When recommen-
evaluating evidence related to drugs, devices, and pro- dationsatLOECaresupportedbyhistoricalclinicaldata,
cedures for detection, management, and prevention of appropriate references (includingclinicalreviews)are cited
disease. When properly applied, expert analysis of avail- ifavailable.Forissueswithsparseavailabledata,asurveyof
able data on the benefits and risks of these therapies and current practice among the clinician members of the
procedures can improve the quality of care, optimize pa- writing committee is the basis for LOE C recommenda-
tient outcomes, and favorably affect costs by focusing tions and no references are cited.
resources on the most effective strategies. An organized Anewadditiontothismethodologyisseparationofthe
and directed approach to a thorough review of evidence Class III recommendations to delineate whether the
has resulted in the production of clinical practice guide- recommendation is determined to be of “no benefit” or is
lines that assist clinicians in selecting the best manage- associated with “harm” to the patient. In addition, in view
mentstrategyforanindividualpatient.Moreover,clinical of the increasing number of comparative effectiveness
practice guidelines can provide a foundation for other studies,comparatorverbsandsuggestedphrasesforwriting
applications, such as performance measures, appropriate recommendations for the comparative effectiveness of one
use criteria, and both quality improvement and clinical treatmentorstrategyversusanotherareincludedforCOR
decision support tools. I and IIa, LOE A or B only.
The American College of Cardiology (ACC) and the In view of the advances in medical therapy across the
American Heart Association (AHA) have jointly engaged spectrum of cardiovascular diseases, the Task Force has
intheproductionofguidelinesintheareaofcardiovascular designated the term guideline-directed medical therapy
disease since 1980. The ACC/AHA Task Force on (GDMT) to represent optimal medical therapy as
Practice Guidelines (Task Force) directs this effort by defined by ACC/AHA guideline (primarily Class I)-
developing, updating, and revising practice guidelines for recommended therapies. This new term, GDMT, is
cardiovascular diseases and procedures. used herein and throughout subsequent guidelines.
Experts in the subject under consideration are selected Because the ACC/AHA practice guidelines address
from both ACC and AHA to examine subject-specific patient populations (and clinicians) residing in North
data and write guidelines. Writing committees are spe- America, drugs that are not currently available in North
cifically charged with performing a literature review; America are discussed in the text without aspecificCOR.
weighing the strength of evidence for or against particular Forstudiesperformedinlargenumbersofsubjectsoutside
tests,treatments,orprocedures;andincludingestimatesof North America, each writing committee reviews the po-
expected health outcomes where such data exist. Patient- tential impact of different practice patterns and patient
specific modifiers, comorbidities, and issues of patient populations on the treatment effect and relevance to the
preference that may influence the choice of tests or ther- ACC/AHA target population to determine whether the
apiesareconsidered,aswellasfrequencyoffollow-upand findings should inform a specific recommendation.
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Table1. ApplyingClassificationofRecommendationsandLevelofEvidence
ArecommendationwithLevelofEvidenceBorCdoesnotimplythattherecommendationisweak.Manyimportantclinicalquestionsaddressedintheguidelinesdonotlendthemselvestoclinicaltrials.
Althoughrandomizedtrialsareunavailable,theremaybeaveryclearclinicalconsensusthataparticulartestortherapyisusefuloreffective.
*Dataavailablefromclinicaltrialsorregistriesabouttheusefulness/efficacyindifferentsubpopulations,suchassex,age,historyofdiabetesmellitus,historyofpriormyocardialinfarction,historyof
heartfailure,andprioraspirinuse.
yForcomparative-effectivenessrecommendations(ClassIandIIa;LevelofEvidenceAandBonly),studiesthatsupporttheuseofcomparatorverbsshouldinvolvedirectcomparisonsofthetreatments
orstrategiesbeingevaluated.
The ACC/AHA practice guidelines are intended to decisions, the goal should be improvement in quality of
assist clinicians in clinical decision making by describinga care. The Task Force recognizes that situations arise in
range of generally acceptable approaches to the diagnosis, which additional data are needed to inform patient care
management, and prevention of specific diseases or con- more effectively; these areas are identified within each
ditions. The guidelines attempt to define practices that respective guideline when appropriate.
meet the needs of most patients in most circumstances. Prescribedcoursesoftreatmentinaccordancewiththese
The ultimate judgment about care of a particular patient recommendations are effective only if followed. Because
mustbemadebytheclinicianandpatientinlightofallthe lackofpatientunderstandingandadherencemayadversely
circumstances presented by that patient. As a result, situ- affect outcomes, clinicians should make every effort to
ations may arise in which deviationsfrom these guidelines engage the patient’s active participation in prescribed
may be appropriate. Clinical decision making should medical regimens and lifestyles. In addition, patients
involve consideration of the quality and availability of should be informed of the risks, benefits, and alternatives
expertise in the area where care is provided. When these to a particular treatment and should be involved in shared
guidelines are used as the basis for regulatory or payer decision making whenever feasible, particularly for COR
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IIa and IIb, for which the benefit-to-risk ratio may be The recommendations in this guideline are considered
lower. current until they are superseded by a focused update,
The Task Force makes every effort to avoid actual, po- the full-text guideline is revised, or until a published
tential,orperceivedconflictsofinterestthatmayariseasa addendum declares it out of date and no longer official
result of relationships with industry and other entities ACC/AHA policy. The reader is encouraged to consult
(RWI) amongthe members of the writing committee.All the full-text guideline (4) for additional guidance and
writing committee members and peer reviewers of the details about valvular heart disease (VHD), since the ex-
guideline are required to disclose all current healthcare- ecutive summary contains only the recommendations.
related relationships, including those existing 12 months Jeffrey L. Anderson, MD, FACC, FAHA
before initiation of the writing effort. Chair, ACC/AHA Task Force on Practice Guidelines
InDecember2009,theACCandAHAimplementeda
newRWIpolicythatrequiresthewritingcommitteechair 1. Introduction
plus a minimum of 50% of the writing committee to
have no relevant RWI (Appendix 1 includes the ACC/
1.1. Methodology and Evidence Review
AHA definition of relevance). The Task Force and all
writing committee members review their respective RWI The recommendations listed in this document are,
disclosures during each conference call and/or meeting of whenever possible, evidence based. An extensive review
the writing committee, and members provide updates to wasconductedonliteraturepublishedthroughNovember
their RWI as changes occur. All guideline recommenda- 2012, and other selected references through October
tions require a confidential vote by the writing committee 2013 were reviewed by the guideline writing committee.
and require approval by a consensus of the voting mem- The relevant data are included in evidence tables in the
bers. Authors’ and peer reviewers’ RWI pertinent to this Data Supplement. Searches were extended to studies,
guideline are disclosed in Appendixes 1 and 2. Members reviews,andotherevidenceconductedonhumansubjects
may not draft or vote on any recommendations and that were published in English from PubMed,
pertaining to their RWI. Members who recused them- EMBASE, Cochrane, Agency for Healthcare Research
selves from voting are indicated in the list of writing and Quality Reports, and other selected databases rele-
committee members with specific section recusals noted vanttothisguideline.Keysearchwordsincludedbutwere
in Appendix 1. In addition, to ensure complete trans- not limited to the following: valvular heart disease, aortic
parency, writing committee members’ comprehensive stenosis, aortic regurgitation, bicuspid aortic valve, mitral
disclosure informationdincluding RWI not pertinent to stenosis, mitral regurgitation, tricuspid stenosis, tricuspid
this documentdis available as an online supplement. regurgitation, pulmonic stenosis, pulmonic regurgitation,
Comprehensive disclosure information for the Task prosthetic valves, anticoagulation therapy, infective endo-
Force is also available online at http://www.cardiosource. carditis, cardiac surgery, and transcatheter aortic valve
org/en/ACC/About-ACC/Who-We-Are/Leadership/ replacement. Additionally, the committee reviewed docu-
Guidelines-and-Documents-Task-Forces.aspx. The ACC ments related to the subject matter previously published
and AHA exclusively sponsor the work of the writing by the ACC and AHA. The references selected and
committee without commercial support. Writing com- published in this document are representative and not
mittee members volunteered their time for this activity. all-inclusive.
Guidelines are official policy of both theACC and AHA.
1.2. Organization of the Writing Committee
In an effort to maintain relevance at the point of care
for clinicians, the Task Force continues to oversee an The committee was composed of clinicians, who included
ongoingprocessimprovementinitiative.Asaresult,several cardiologists, interventionalists, surgeons, and anesthesi-
changes to these guidelines will be apparent, including ologists. The committee included representatives from
limited narrative text, a focus on summary and evidence theAmericanAssociationforThoracicSurgery,American
tables(withreferenceslinkedtoabstractsinPubMed),and Society of Echocardiography (ASE), Society for Cardio-
more liberal use of summary recommendation tables (with vascular Angiography and Interventions, Society of Car-
referencesthatsupportLOE)toserveasaquickreference. diovascular Anesthesiologists, and Society of Thoracic
In April 2011, the Institute of Medicine released 2 Surgeons (STS).
reports: Finding What Works in Health Care: Standards
1.3. Document Review and Approval
for Systematic Reviews and Clinical Practice Guidelines
We Can Trust (2,3). It is noteworthy that the Institute This document was reviewed by 2 official reviewers each
of Medicine cited ACC/AHA practice guidelines as be- nominated by both the ACC and the AHA, as well as 1
ing compliant with many of the proposed standards. reviewereachfromtheAmericanAssociationforThoracic
A thorough review of these reports and of our current Surgery,ASE,SocietyforCardiovascularAngiographyand
methodology is under way, with further enhancements Interventions, Society of Cardiovascular Anesthesiologists,
anticipated. and STS and 39 individual content reviewers (which
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included representatives from the following ACC com- up, 2) medical therapy, and 3) intervention. The purpose
mittees and councils: Adult Congenital and Pediatric of these subsections was to categorize the COR according
Cardiology Section, Association of International Gover- to the clinical decision-making pathways that caregivers
nors, Council on Clinical Practice, Cardiovascular Section use in the management of patients with VHD. New rec-
Leadership Council, Geriatric Cardiology Section Lead- ommendations for assessment of the severity of valve le-
ership Council, Heart Failure and Transplant Council, sionshavebeenproposed,basedoncurrentnaturalhistory
Interventional Council, Lifelong Learning Oversight studies of patients with VHD. The relevant data are
Committee, Prevention of Cardiovascular Disease Com- includedin evidencetablesintheData Supplementof the
mittee, and Surgeon Council). Reviewers’ RWI informa- full-text guideline (4).
tion was distributed to the writing committee and is The present document applies to adult patients with
published in this document (Appendix 2). VHD. Management of patients with congenital heart
This document was approved for publication by the disease(CHD)andinfantsandchildrenwithvalvedisease
governingbodiesoftheACCandtheAHAandendorsed are not addressed here. The document recommends a
by the American Association for Thoracic Surgery, ASE, combination of lifestyle modifications and medications
SocietyforCardiovascularAngiographyandInterventions, that constitute GDMT. Both for GDMT and other rec-
Society of Cardiovascular Anesthesiologists, and STS. ommended drugtreatmentregimens, thereaderis advised
to confirm dosages with product insert material and to
carefully evaluate for contraindications and drug–drug in-
1.4. Scope of the Guideline
teractions.Table2isalistofassociatedguidelinesthatmay
The focus of this guideline is the diagnosis and manage- beofinteresttothereader.Thetableisintendedforuseas
ment of adult patients with valvular heart disease (VHD). aresourceandobviates theneedtorepeatextantguideline
A full revision of the original 1998 VHD guideline was recommendations.
madein2006,andanupdatewasmadein2008(5).Some
recommendations from the earlier VHD guidelines have 2. General Principles
been updated as warranted by new evidence or a better
understanding of earlier evidence, whereas others that
2.1. Evaluation of the Patient With
were inaccurate, irrelevant, or overlapping were deleted or
modified. Throughout, our goal was to provide the clini- Suspected VHD
cian with concise, evidence-based, contemporary recom- Patients with VHD may present with a heart murmur,
mendations and the supporting documentation to symptoms, or incidental findings of valvular abnormalities
encourage their use. onchestimagingornoninvasivetesting.Irrespectiveofthe
The full-textversionofthisguideline(4) wascreatedin presentation, all patients with known or suspected VHD
a different format from prior VHD guidelines to facilitate should undergo an initial meticulous history and physical
access to concise, relevant bytes of information at the examination,aswellasachestx-rayandelectrocardiogram.
point of care when clinical knowledge is needed the most. A comprehensive transthoracic echocardiogram (TTE)
Thus, each COR is followed by a brief paragraph of sup- with 2-dimensional imaging and Doppler interrogation
porting text and references. Where applicable, sections should then be performed to correlate findings with
were divided into subsections of 1) diagnosis and follow- initial impressions based on the initial clinical evaluation.
Table2. AssociatedGuidelinesandStatements
Publication
Title Organization Year/Reference
RecommendationsforEvaluationoftheSeverityofNativeValvularRegurgitationWithTwo-Dimensionaland ASE 2003(6)
DopplerEchocardiography
GuidelinesfortheManagementofAdultsWithCongenitalHeartDisease ACC/AHA 2008(8)
EchocardiographicAssessmentofValveStenosis:EAE/ASERecommendationsforClinicalPractice EAE/ASE 2009(9)
RecommendationsforEvaluationofProstheticValvesWithEchocardiographyandDopplerUltrasound ASE 2009(10)
GuidelinefortheDiagnosisandTreatmentofHypertrophicCardiomyopathy ACCF/AHA 2011(11)
GuidelinesontheManagementofCardiovascularDiseasesDuringPregnancy ESC 2011(12)
AntithromboticandThrombolyticTherapyforValvularDisease:AntithromboticTherapyandPreventionofThrombosis ACCP 2012(13)
GuidelinesontheManagementofValvularHeartDisease ESC/EACTS 2012(14)
GuidelinefortheManagementofHeartFailure ACCF/AHA 2013(15)
GuidelinefortheManagementofPatientsWithAtrialFibrillation AHA/ACC/HRS 2014(16)
ACCindicatesAmericanCollegeofCardiology;ACCF,AmericanCollegeofCardiologyFoundation;ACCP,AmericanCollegeofChestPhysicians;AF,atrialfibrillation;AHA,AmericanHeartAssociation;
ASE,AmericanSocietyofEchocardiography;EACTS,EuropeanAssociationforCardio-ThoracicSurgery;EAE,EuropeanAssociationofEchocardiography;ESC,EuropeanSocietyofCardiology;andVHD,
valvularheartdisease.
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The TTE will also be able to provide additional infor- Table3. StagesofProgressionofVHD
mation, such as the effect of the valve lesion on
Stage Definition Description
the cardiac chambers and great vessels, and to assess for
A Atrisk PatientswithriskfactorsfordevelopmentofVHD
other concomitant valve lesions. Other ancillary testing
B Progressive PatientswithprogressiveVHD(mild-to-moderate
such as transesophageal echocardiography (TEE), com- severityandasymptomatic)
puted tomography (CT) or cardiac magnetic resonance C Asymptomatic Asymptomaticpatientswhohavethecriteriafor
(CMR) imaging, stress testing, and diagnostic hemody- severe severeVHD:
namic cardiac catheterization may be required to deter- C1:AsymptomaticpatientswithsevereVHDinwhom
theleftorrightventricleremainscompensated
mine the optimal treatment for a patient with VHD. An
C2:AsymptomaticpatientswithsevereVHD,with
evaluationofthepossiblesurgicalriskforeachindividual
decompensationoftheleftorrightventricle
patient should be performed if intervention is contem- D Symptomatic Patientswhohavedevelopedsymptomsasaresult
plated, as well as other contributing factors such as the severe ofVHD
presence and extent of comorbidities and frailty. Follow- VHDindicatesvalvularheartdisease.
up of these patients is important and should consist
of an annual history and physical examination in most
stable patients. An evaluation of the patient may be to measurements of severity. In patients with stenotic le-
necessary sooner than annually if there is a change in the sions, there is an additional category of “very severe” ste-
patient’s symptoms. In some valve lesions there may be nosis based on studies of the natural history showing that
unpredictable adverse consequences on the left ventricle prognosis becomes poorer as the severity of stenosis
in the absence of symptoms necessitating more frequent increases.
follow-up. The frequency of repeat testing, such as
echocardiography, will be dependent on the severity of 2.3. Diagnostic TestingdDiagnosis and
thevalvelesionanditseffectontheleftorrightventricle, Follow-Up: Recommendations
coupled with the known natural history of the valve
See Table 4 for the frequency of echocardiograms in
lesion.
asymptomatic patients with VHD and normal left ven-
2.2. Definitions of Severity of Valve Disease tricular (LV) function.
Classification of the severity of valve lesions should be CLASSI
based on multiple criteria, including the initial findings 1. TTE is recommended in the initial evaluation of patients with
known or suspected VHD to confirm the diagnosis, establish
on the physical examination, which should then be corre-
etiology, determine severity, assess hemodynamic conse-
lated with data from a comprehensive TTE. Intervention
quences, determine prognosis, and evaluate for timing of
should primarily be performed on patients with severe
intervention(19–34).(LevelofEvidence:B)
VHD in addition to other criteria outlined in this
2. TTE is recommended in patients with known VHD with any
document. changeinsymptomsorphysicalexaminationfindings.(Levelof
This document provides a classification of the progres- Evidence:C)
sion of VHD with 4 stages (A to D) similar to that pro- 3. PeriodicmonitoringwithTTEisrecommendedinasymptomatic
posed by the “2013 ACCF/AHA Guideline for the patientswithknownVHDatintervalsdependingonvalvelesion,
Management of Heart Failure” (18). Indication for inter- severity, ventricular size, and ventricular function. (Level of
Evidence:C)
vention in patients with VHD is dependent on 1) the
4. Cardiac catheterization for hemodynamic assessment is rec-
presenceorabsenceofsymptoms;2)theseverityofVHD;
ommendedinsymptomaticpatientswhennoninvasivetestsare
3) the response of the left and/or right ventricle to the
inconclusiveorwhenthereisadiscrepancybetweenthefind-
volume or pressure overload causedby VHD; 4)the effect
ingsonnoninvasivetestingandphysicalexaminationregarding
on the pulmonary or systemic circulation; and 5) a change
severityofthevalvelesion.(LevelofEvidence:C)
in heart rhythm. The stages take into consideration all of
theseimportantfactors(Table3).Thecriteriaforthestages CLASSIIa
1. Exercisetestingisreasonableinselectedpatientswithasymp-
of each individual valve lesion are listed in Section 3.1,
tomaticsevereVHDto1)confirmtheabsenceofsymptoms,or
Section 4.1, Section 6.1, Section 7.1, Section 8.1, Section
2)assessthehemodynamicresponsetoexercise,or3)determine
8.3,and Section 9. prognosis(35–39).(LevelofEvidence:B)
The purpose of valvular intervention is to improve
symptoms and/or prolong survival, as well as to minimize
the risk of VHD-related complications such as asymp- 2.4. Basic Principles of Medical Therapy:
tomatic irreversible ventricular dysfunction, pulmonary Recommendations
hypertension,stroke,andatrialfibrillation(AF).Thus,the
CLASSI
criteria for “severe” VHD are based on studies describing 1. Secondarypreventionofrheumaticfeverisindicatedinpatients
the natural history of patients with unoperated VHD, as withrheumaticheartdisease,specificallymitralstenosis(MS)
wellasobservationalstudiesrelatingtheonsetofsymptoms (40).(LevelofEvidence:C)
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Table4. FrequencyofEchocardiogramsinAsymptomaticPatientsWithVHDandNormalLeftVentricularFunction
Stage ValveLesion
Stage AorticStenosis* AorticRegurgitation MitralStenosis MitralRegurgitation
Progressive Every3–5y Every3–5y(mildseverity) Every3–5y Every3–5y(mildseverity)
(stageB) (mildseverityV 2.0–2.9m/s) Every1–2y(moderateseverity) (MVA>1.5cm2) Every1–2y(moderateseverity)
max
Every1–2y
(moderateseverityV 3.0–3.9m/s)
max
Severe Every6–12mo Every6–12mo Every1–2y Every6–12mo
(stageC) (V (cid:3)4m/s) DilatingLV:morefrequently (MVA1.0–1.5cm2) DilatingLV:morefrequently
max
Onceeveryyear
(MVA<1.0cm2)
Patientswithmixedvalvediseasemayrequireserialevaluationsatintervalsearlierthanrecommendedforsinglevalvelesions.
*Withnormalstrokevolume.
LVindicatesleftventricle;MVA,mitralvalvearea;VHD,valvularheartdisease;andVmax,maximumvelocity.
Table5. RiskAssessmentCombiningSTSRiskEstimate,Frailty,MajorOrganSystemDysfunction,
andProcedure-SpecificImpediments
LowRisk IntermediateRisk HighRisk ProhibitiveRisk
(MustMeetALLCriteria (Any1Criterion (Any1Criterion (Any1Criterion
inThisColumn) inThisColumn) inThisColumn) inThisColumn)
STSPROM* <4% 4%–8% >8% Predictedriskwithsurgeryofdeath
AND OR OR ormajormorbidity(all-cause)
Frailtyy None 1Index(mild) (cid:3)2Indices(moderatetosevere) >50%at1y
AND OR OR OR
Majororgansystemcompromise None 1Organsystem Nomorethan2organsystems (cid:3)3Organsystems
nottobeimprovedpostoperativelyz AND OR OR OR
Procedure-specificimpedimentx None Possibleprocedure-specific Possibleprocedure-specific Severeprocedure-specific
impediment impediment impediment
*UseoftheSTSPROMtopredictriskinagiveninstitutionwithreasonablereliabilityisappropriateonlyifinstitutionaloutcomesarewithin1standarddeviationofSTSaverageobserved/expectedratio
fortheprocedureinquestion.
ySevenfrailtyindices:KatzActivitiesofDailyLiving(independenceinfeeding,bathing,dressing,transferring,toileting,andurinarycontinence)andindependenceinambulation(nowalkingaidorassist
requiredor5-meterwalkin<6s).Otherscoringsystemscanbeappliedtocalculateno,mild-,ormoderate-to-severefrailty.
zExamplesofmajororgansystemcompromise:CardiacdsevereLVsystolicordiastolicdysfunctionorRVdysfunction,fixedpulmonaryhypertension;CKDstage3orworse;pulmonarydysfunctionwith
FEV1<50%orDLCO2<50%ofpredicted;CNSdysfunction(dementia,Alzheimer’sdisease,Parkinson’sdisease,CVAwithpersistentphysicallimitation);GIdysfunctiondCrohn’sdisease,ulcerative
colitis,nutritionalimpairment,orserumalbumin<3.0;cancerdactivemalignancy;andliverdanyhistoryofcirrhosis,varicealbleeding,orelevatedINRintheabsenceofVKAtherapy.
xExamples:tracheostomypresent,heavilycalcifiedascendingaorta,chestmalformation,arterialcoronarygraftadherenttoposteriorchestwall,orradiationdamage.
CKDindicateschronickidneydisease;CNS,centralnervoussystem;CVA,stroke;DLCO2,diffusioncapacityforcarbondioxide;FEV1,forcedexpiratoryvolumein1s;GI,gastrointestinal;INR,
internationalnormalizedratio;LV,leftventricular;PROM,predictedriskofmortality;RV,rightventricular;STS,SocietyofThoracicSurgeons;andVKA,vitaminKantagonist.
CLASSIIa 2.5. Evaluation of Surgical and
1. Prophylaxis against infective endocarditis (IE) is reasonable Interventional Risk
forthe followingpatientsathighest riskforadverse outcomes
See Table 5 for risk assessment combining STS risk esti-
from IE before dental procedures that involve manipulation
mate, frailty, major organ system dysfunction, and
ofgingivaltissue,manipulationoftheperiapicalregionofteeth,or
perforationoftheoralmucosa(41–43)(LevelofEvidence:B): procedure-specific impediments.
(cid:2) Patientswithprostheticcardiacvalves;
2.6. The Heart Valve Team and Heart Valve
(cid:2) PatientswithpreviousIE;
Centers of Excellence: Recommendations
(cid:2) Cardiactransplantrecipientswithvalveregurgitationdueto
astructurallyabnormalvalve;or CLASSI
(cid:2) PatientswithCHDwith: 1. PatientswithsevereVHDshouldbeevaluatedbyamultidisci-
B UnrepairedcyanoticCHD,includingpalliativeshuntsand plinary Heart Valve Team when intervention is considered.
(LevelofEvidence:C)
conduits;
B Completelyrepairedcongenitalheartdefectrepairedwith
CLASSIIa
prostheticmaterialordevice,whetherplacedbysurgery 1. ConsultationwithorreferraltoaHeartValveCenterofExcel-
or catheter intervention, during the first 6 months after lence is reasonable when discussing treatment options for 1)
theprocedure;or asymptomaticpatientswithsevereVHD,2)patientswhomay
B Repaired CHD with residual defects at the site or adja- benefit from valve repair versus valve replacement, or 3)
centtothesiteofaprostheticpatchorprostheticdevice. patientswithmultiplecomorbiditiesforwhomvalveintervention
CLASSIII:NoBenefit isconsidered.(LevelofEvidence:C)
1. Prophylaxis against IE is not recommended in patients with
VHDwhoareatriskofIEfornondentalprocedures(e.g.,TEE, A competent, practicing cardiologist should have the
esophagogastroduodenoscopy, colonoscopy, or cystoscopy) abilitytodiagnoseanddirectthetreatmentofmostpatients
intheabsenceofactiveinfection(44).(LevelofEvidence:B) with VHD. For instance, otherwise healthy patients with
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severeVHDwhobecomesymptomaticshouldnearlyalways 3.1. Stages of Valvular AS
be considered for intervention. However, more complex
Medicalandinterventionalapproachestothemanagement
decision-making processes may be required in select
of patients with valvular AS depend on accurate diagnosis
patientpopulations,suchasthosewhohaveasymptomatic
ofthecauseandstageofthediseaseprocess.Table6shows
severe VHD, those who are at high risk for intervention,
thestagesofASrangingfrompatientsatriskofAS(stage
or those who could benefit from specialized therapies
A)orwithprogressivehemodynamicobstruction(stageB)
suchasvalverepairortranscathetervalveintervention.
to severe asymptomatic (stage C) and symptomatic AS
ThemanagementofpatientswithcomplexsevereVHD (stageD).Eachofthesestagesisdefinedbyvalveanatomy,
is best achieved by a Heart Valve Team composed pri-
valvehemodynamics,theconsequencesofvalveobstruction
marilyofacardiologistandsurgeon(includingastructural
on the left ventricle and vasculature, as well as by patient
valve interventionist if a catheter-based therapy is being
symptoms. Hemodynamic severity is best characterized by
considered). In selected cases, there may be a multidisci-
the transaortic maximum velocity (or mean pressure
plinary, collaborative group of caregivers, including cardi- gradient) when the transaortic volume flow rateis normal.
ologists, structural valve interventionalists, cardiovascular
However, some patients with AS have a low transaortic
imaging specialists, cardiovascular surgeons, anesthesiolo- volume flow rate due to either LV systolic dysfunction
gists, and nurses, all of whom have expertise in the man-
withalowleftventricularejectionfraction(LVEF)ordue
agement and outcomes of patients with complex VHD.
to a small hypertrophied left ventricle with a low stroke
The Heart Valve Team should optimize patient selection
volume. These categories of severe AS pose a diagnostic
for available procedures through a comprehensive under-
and management challenge distinctly different from the
standing of the risk–benefit ratio of different treatment
challenges faced by the majority of patients with AS, who
strategies.Thisisparticularlybeneficialinpatientsinwhom
haveahighgradientandvelocitywhenASissevere.These
there are several options for treatment, such as the elderly special subgroups with low-flow AS are designated D2
high-risk patient with severe symptomatic aortic stenosis
(with alow LVEF) and D3 (with anormal LVEF).
(AS) being considered for transcatheter aortic valve re- The definition of severe AS is based on natural history
placement (TAVR) or surgical aortic valve replacement
studies of patients with unoperated AS, which show that
(AVR). The patient and family should be sufficiently
the prognosis is poor once there is a peak aortic valve ve-
educated by the Heart Valve Team about all alternatives locity of >4.0 m per second, corresponding to a mean
fortreatmentsothat theirexpectationscanbemetasfully aortic valve gradient >40 mm Hg. In patients with low
aspossibleusingashareddecision-makingapproach. forward flow, severe AS can be present with lower aortic
The optimal care of the patient with complex heart
valve velocities and lower aortic valve gradients. Thus, an
disease is best performed in centers that can provide all
aorticvalveareashouldbecalculatedinthesepatients.The
availableoptionsfordiagnosisandmanagement,including
prognosis of patients with AS is poorer when the aortic
theexpertiseforcomplexaorticormitralvalverepair,aortic valveareais<1.0cm2.Atnormalflowrates,anaorticvalve
surgery, and transcatheter therapies. This has led to the area of <0.8 cm2 correlates with a mean aortic valve
development of Heart Valve Centers of Excellence. Heart gradient >40 mm Hg. However, symptomatic patients
Valve Centers of Excellence 1) are composed of experi- with a calcified aortic valve with reduced opening and an
enced healthcare providers with expertise from multiple aortic valve area between 0.8 cm2 and 1.0 cm2 should be
disciplines; 2) offer all available options for diagnosis and closely evaluated to determine whether they would benefit
management, including complex valve repair, aortic sur-
from valve intervention. Meticulous attention to detail is
gery, and transcatheter therapies; 3) participate in regional
requiredwhenassessingaorticvalvehemodynamics,either
ornationaloutcomeregistries;4)demonstrateadherenceto
with Doppler echocardiography or cardiac catheterization,
national guidelines; 5) participate in continued evaluation
and the inherent variability of the measurements and cal-
and quality improvement processes to enhance patient
culations should always be considered in clinical-decision
outcomes; and 6) publicly report their available mortality
making.
and success rates. Decisions about intervention at the
HeartValveCentersofExcellenceshouldbedependenton
the centers’ publicly available mortality rates and operative 3.2. Diagnosis and Follow-Up
outcomes. It is recognized that some Heart Valve Centers The overall approach to the initial diagnosis of VHD is
of Excellence may have expertise in select valve problems. discussed in Section 2.3, and additional considerations
specific to patients with AS are addressed here.
3. Aortic Stenosis: Recommendations
CLASSI
1. TTE is indicated in patients with signs or symptoms of AS or
SeeTable6forthestagesofvalvularAS;Tables7and8for
a bicuspid aortic valve for accurate diagnosisof the cause of
a summary of recommendations for choice and timing of AS,hemodynamicseverity,LVsize,andsystolicfunction,and
intervention; and Figure 1 for indications for AVR in pa- for determining prognosis and timing of valve intervention
tients with AS. (26,27,45).(LevelofEvidence:B)
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Table6. StagesofValvularAS
Stage Definition ValveAnatomy ValveHemodynamics HemodynamicConsequences Symptoms
A AtriskofAS (cid:2) Bicuspidaorticvalve(orother (cid:2) AorticV <2m/s (cid:2) None (cid:2) None
max
congenitalvalveanomaly)
(cid:2) Aorticvalvesclerosis
B ProgressiveAS (cid:2) Mild-to-moderateleaflet (cid:2) MildAS: (cid:2) EarlyLVdiastolic (cid:2) None
calcificationofabicuspid AorticV 2.0–2.9m/sor dysfunctionmay
max
ortrileafletvalvewith meanDP<20mmHg bepresent
somereductioninsystolic (cid:2) ModerateAS: (cid:2) NormalLVEF
motionor AorticV 3.0–3.9m/sor
max
(cid:2) Rheumaticvalvechanges meanDP20–39mmHg
withcommissuralfusion
C:AsymptomaticsevereAS
C1 Asymptomaticsevere (cid:2) Severeleafletcalcification (cid:2) AorticV (cid:3)4m/sor (cid:2) LVdiastolic (cid:2) None:Exercise
max
AS orcongenitalstenosiswith meanDP(cid:3)40mmHg dysfunction testingis
severelyreducedleaflet (cid:2) AVAtypicallyis(cid:4)1.0cm2 (cid:2) MildLVhypertrophy reasonableto
opening (orAVAi(cid:4)0.6cm2/m2) (cid:2) NormalLVEF confirm
(cid:2) VerysevereASisanaortic symptomstatus
V (cid:3)5m/sormean
max
DP(cid:3)60mmHg
C2 Asymptomaticsevere (cid:2) Severeleafletcalcification (cid:2) AorticV (cid:3)4m/sor (cid:2) LVEF<50% (cid:2) None
max
ASwithLV orcongenitalstenosiswith meanDP(cid:3)40mmHg
dysfunction severelyreducedleaflet (cid:2) AVAtypically(cid:4)1.0cm2
opening (orAVAi(cid:4)0.6cm2/m2)
D:SymptomaticsevereAS
D1 Symptomaticsevere (cid:2) Severeleafletcalcification (cid:2) AorticV (cid:3)4m/sor (cid:2) LVdiastolic (cid:2) Exertional
max
high-gradientAS orcongenitalstenosiswith meanDP(cid:3)40mmHg dysfunction dyspneaor
severelyreducedleaflet (cid:2) AVAtypically(cid:4)1.0cm2 (cid:2) LVhypertrophy decreasedexer-
opening (orAVAi(cid:4)0.6cm2/m2)butmay (cid:2) Pulmonary cisetolerance
belargerwithmixedAS/AR hypertensionmay (cid:2) Exertionalangina
bepresent (cid:2) Exertional
syncopeor
presyncope
D2 Symptomaticsevere (cid:2) Severeleafletcalcification (cid:2) AVA(cid:4)1.0cm2with (cid:2) LVdiastolic (cid:2) HF
low-flow/low- withseverelyreducedleaflet restingaorticV <4m/sor dysfunction (cid:2) Angina
max
gradientASwith motion meanDP<40mmHg (cid:2) LVhypertrophy (cid:2) Syncopeor
reducedLVEF (cid:2) Dobutaminestressechocardiography (cid:2) LVEF<50% presyncope
showsAVA(cid:4)1.0cm2with
V (cid:3)4m/satanyflowrate
max
D3 Symptomaticsevere (cid:2) Severeleafletcalcification (cid:2) AVA(cid:4)1.0cm2withaorticV (cid:2) IncreasedLVrelative (cid:2) HF
max
low-gradientAS withseverelyreducedleaflet <4m/sormeanDP<40mmHg wallthickness (cid:2) Angina
withnormalLVEFor motion (cid:2) IndexedAVA(cid:4)0.6cm2/m2and (cid:2) SmallLVchamber (cid:2) Syncopeor
paradoxicallow-flow (cid:2) Strokevolumeindex<35mL/m2 withlowstrokevolume presyncope
severeAS (cid:2) Measuredwhenpatientis (cid:2) Restrictivediastolic
normotensive(systolic filling
BP<140mmHg) (cid:2) LVEF(cid:3)50%
ARindicatesaorticregurgitation;AS,aorticstenosis;AVA,aorticvalvearea;AVAi,aorticvalveareaindexedtobodysurfacearea;BP,bloodpressure;HF,heartfailure;LV,leftventricular;LVEF,left
ventricularejectionfraction;DP,pressuregradient;andVmax,maximumaorticvelocity.
CLASSIIa CLASSIII:Harm
1. Low-dosedobutaminestresstestingusingechocardiographicor 1. Exercisetestingshouldnotbeperformedinsymptomaticpatients
invasivehemodynamicmeasurementsisreasonableinpatients withASwhentheaorticvelocityis4.0mpersecondorgreateror
with stage D2 AS with all of the following (46–48) (Level of meanpressuregradientis40mmHgorhigher(stageD) (50).
Evidence:B): (LevelofEvidence:B)
a. Calcifiedaorticvalvewithreducedsystolicopening;
b. LVEFlessthan50%;
3.3. Medical Therapy
c. Calculatedvalvearea1.0cm2orless;and
d. Aorticvelocitylessthan4.0mpersecondormeanpressure CLASSI
gradientlessthan40mmHg. 1. Hypertension in patients at risk for developing AS
2. Exercisetestingisreasonabletoassessphysiologicalchanges (stageA)andinpatientswithasymptomaticAS(stagesB
with exercise and to confirm the absence of symptoms in and C) should be treated according to standard GDMT,
asymptomaticpatientswithacalcifiedaorticvalveandanaortic started at a low dose, and gradually titrated upward as
velocity4.0mpersecondorgreaterormeanpressuregradient40 needed with frequent clinical monitoring (51–53). (Level of
mmHgorhigher(stageC)(27,37,38,49).(LevelofEvidence:B) Evidence:B)
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