Adapted from the 2011 ACCF/AHA Guideline for the Diagnosis ...

ACCF/AHA Pocket Guideline

Adapted from the 2011 ACCF/AHA

Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy

November 2011

Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

? 2011 by the American College of Cardiology Foundation and the American Heart Association, Inc.

The following material was adapted from the 2011 ACCF/AHA Guidelines for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (J Am Coll Cardiol 2011;XX:XX?XX). This pocket guideline is available on the World Wide Web sites of the American College of Cardiology (cardiosource. org) and the American Heart Association (my.).

For copies of this document, please contact Elsevier Inc. Reprint Department, e-mail: reprints@; phone: 212-633-3813; fax: 212-633-3820.

Permissions: Multiple copies, modification, alteration, enhancement, and/ ordistribution of this document are not permitted without the express permission of the American College of Cardiology Foundation. Please contact Elsevier's permission department at healthpermissions@.

Contents

1. Introduction..........................................................................................3 2. Clinical Definition.................................................................................6 3. Genetic Testing Strategies/Family Screening......................................7 4. Genotype-Positive/Phenotype-Negative Patients..............................9 5. Echocardiography............................................................................... 10 6. Stress Testing...................................................................................... 14 7. Cardiac Magnetic Resonance............................................................. 15 8. Detection of Concomitant Coronary Disease.................................... 17 9. Asymptomatic Patients...................................................................... 19 10. Pharmacologic Management............................................................ 21 11. Invasive Therapies............................................................................ 26

12. Pacing............................................................................................... 29 13. Sudden Cardiac Death Risk Stratification...................................... 30 14. Selection of Patients for Implantable Cardioverter-Defibrillators....32 15. Participation in Competitive or Recreational Sports

and Physical Activity....................................................................... 36 16. Management of Atrial Fibrillation.................................................. 38 17. Pregnancy/Delivery......................................................................... 41

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1. Introduction

The impetus for the guidelines is based on an appreciation of the frequency of this clinical entity and a realization that many aspects of clinical management, including the use of diagnostic modalities, genetic testing, utilization of implantable cardioverter-defibrillators (ICDs), and therapies for refractory symptoms lack consensus. The discussion and recommendations about the various diagnostic modalities apply to patients with established HCM and to a variable extent to patients with a high index of suspicion of the disease. Classification of Recommendations The ACCF/AHA classifications of recommendations and levels of evidence are utilized, and described in more detail in Table 1.

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Applying ClassTifaicTbaatliebonl1e.oAf1R.pepAclopympilnmyignegnCdClaaltasisossniisffiicacanatdtioiLnoenovefolRofefcEovmidmeenncdeations and Level of Evidence Recommendations and Level of Evidence

S i z e o f T r e a T m eSni Tz ee foffe cT Tr e a T

Class I Benefit >>> Risk

Procedure/Treatment should be performed/ administered

ClCalsasssIIaI

BeBneenfietf>it >>Ri>skRisk AdPdritoicoendaul rsetu/Tdrieeastwmiethnt focsuhsoedulodbjbeectipveersfonremeedde/d ItaIdsmrienaissteorneadble to perform procedure/administer treatment

ClCalsasssIIbIIa

BeBneenfeitfi>t >R>iskRisk AdAddidtiiotinoanlasltustduideisews iwthithbroad obfjoeccutsiveedsonbejedcetidv;esadndeietidoendal reIgtisItsryrdeaatasownoaubldlbeetohepleprfu- l Prfocrmedupreoc/Terdeuartem/aednmt inister mtareyabtmeeCnot nsIdered

eSTimaTe of cerTainTy (PreciSion) of TreaTmenT effecT

a

populations d*

ived from multiple zed clinical trials analyses

n Rleecvoemlmaendation that procedure or treatment is uMseufltuipl/leeffpeocptiuvleations

evaluated* n Sufficient evidence from muDltaiptaledrearnivdeodmfirzoemd tmriaullstiple or mraentdao-manizaelydscelsinical trials

or meta-analyses

n RneRceocmommemnednadtiaotnioinn tfhaavtor of ptreoactemduernet or ptrreoactemdeunret beisngusuesfeuflu/le/fefeffcetcivtieve

n SnoSmuefficcoiennflticetvinidgeenvciedefrnocme frommulmtipuletiprlaenrdaonmdiozmedizterdials triaolrsmoer tma-eatnaa-alynsaelsyses

n nReRceocmommenednadtaiotnio'ns in favor usoeffutrlenaetsms/eenftfiocracpyrolceesdsure weblel iensgtaubsleisfuhle/deffective

n nGrSeoamteer conflicting evidence evfirdoemncmeuflrtoipmlemraunltdipolme ized ratnrdiaolms oizremd etrtiaa-lasnoarlyses meta-analyses

b

populations d*

ived from a ndomized trial ndomized studies

n Rleecvoemlmbendation that pisrouLcsiemedfiuutler/edefopfreocptrtueivlaaettmioennst

evaluated* n Evidence from single ranDdaotmaidzeedrivtreidalfororm a nonsrianngdleomrainzdedomstiuzeddietsrial

or nonrandomized studies

n RneRceocmommemnednadtiaotnioinn tfhaavtor of ptreoactemduernet or ptrreoactemdeunret beisngusuesfeuflu/le/fefeffcetcivtieve

n SnoEmveidceonncfelicfrtoinmg single evridaenndcoemfirzoemd tsrianlgloer randoonmrainzdeodmtriizaeldosr tudies nonrandomized studies

n nReRceocmommenednadtaiotnio'ns in favor usoeffutrlenaetsms/eenftfiocracpyrolceesdsure weblel iensgtaubsleisfuhle/deffective

n nGrSeoamteer conflicting eveidviednecnecefrofrmomsinsignlgele rarnadnodmomizeizdedtritarilaolror nonnornarnadnodmomizeizdedstustduidesies

C

ited populations d*

sensus opinion ts, case studies, ard of care

n RleecvoemlmCendation that proVceerdyulriemoitretdrepaotpmuelanttioisns useefvual/lueaffteecdt*ive sntuOOodnfinelyelsyx,epcoxeorprnestssrteat, nnocdsapuasinsredioosonptfu,indcciaiaoersnsee,

or standard of care

n RneRceocmommemnednadtiaotnioinn tfhaavtor of ptreoactemduernet or ptrreoactemdeunret is beuinsgefusle/efuffle/cetfifveective

n OnnOlynldyiveexrpgeirntgoepxinpieornt, case opsintuiodnie,sc,aosresstatunddiaersd, of care or standard of care

n nReRceocmommenednadtaiotnio'ns in favor usoeffutrlenaetsms/eenftfiocracpyrolceesdsure weblel iensgtaubsleisfuhle/deffective

n nOnOlnyldyidveivregrignigngexepxepret rt opoipninonio,nc,acsaesestustduidesie,so,r staonr dsatardndoafrcdaorfecare

d phrases for commendations

ive ess phrases

4

shoSuuldggested phrases for is rewcroitmingmreencdoemdmendations is indicated is useful/effective/beneficial

treaCtmomenpta/sratrtaivtegy A is recoemffemcteivnedneeds/isndpihcraatseedsin preference to treatment B treatment A should be chosen over treatment B

is rsehaosuoldnable canisbre cuosmefmule/enfdfeecdtive/beneficial is pisroinbdaibclayteredcommended orisinudsiceafutel/deffective/beneficial

tretartematemnte/nst/rsatreagtyegAyiAs pisrobably recroemcommemndeend/eindd/iincdaitceadteind in prepfreerfeenrceenctoe toretartematemntenBt B it istrereaatmsoennatbAlesthoocuhldoobseechosen treoavtmeretnrteaAtmoveenrt tBreatment B

maisy/rmeaigshotnabbeleconsidered

macya/nmbigehutsbeefurle/eafsfeocntaivbele/beneficial

usiesfuplrnoebsasb/elyffreecctoivmenmeesnsdised unokrnionwdinc/autnecdlear/uncer tain or not well established

s

treatment/strategy A is probably

recommended/indicated in

preference to treatment B

it is reasonable to choose

e

treatment A over treatment B

TmenT effecT

ClCalsasssIII INbo Benefit orBCelnaefsits>IIIRHisakrm

AdditionaPlroscteudduirees/ with broad

test

treatment

objectives needed; additional

CnoorrebegIIniIse: tfrity dHNaoetltpafuwl ould BNbeoenPehrfeoitvlpenful

CoPrroIIcI:edurEexc/eTsrseCaotsmt eHnatrmful hamrmay be wC/ooBnesnIedfiterteodPatients

or Harmful

n nReRceocmommenednadtaiotnionth'sat pruoscefduulnre sosr/terfefiactamceynlteisss nowt eulsleefsutla/ebflfieschteidve and may benhGarremaftuelr conflicting n eSvuifdfeicniceentfreovmidemnucletipfrloem mrualtnipdloemriaznedotmriaizlesdotrrials or mmeteat-a-naanlyaslyesses

Class III No Benefit or Class III Harm

Procedure/

test

treatment

Cor III: Not no benefit Helpful

No Proven Benefit

Cor III: harm

Excess Cost Harmful w/o Benefit to Patients or Harmful

n Recommendation that procedure or treatment is not useful/effective and may be harmful

n Sufficient evidence from multiple randomized trials or meta-analyses

n nReRceocmommenednadtaiotnionth'sat pruoscefduulnre sosr/terfefiactamceynlteisss nowt eulsl eefsutla/ebflfieschteidve and may benhGaremaftuelr conflicting n eEvviiddeennccee ffrroomm ssiinnggllee rarnadnodmomizeizdedtritarliaolror nonnornarnadnodmomizeizdedstustduiedsies

n nReRceocmommmenednadtaiotnionth'sat pruosceefduulnreesosr/terfefiactamceynlteisss nowt eulsl eefsutla/ebflfieschteidve and may benhOanrmlyfudliverging expert n oOpnilnyioenx,pceartseopsitnuidoine,sc, aosre stustdaiensd,aordr sotfacnadraerd of care

n Recommendation that procedure or treatment is not useful/effective and may be harmful n Evidence from single randomized trial or nonrandomized studies

n Recommendation that procedure or treatment is not useful/effective and may be harmful n Only expert opinion, case studies, or standard of care

A recommendation with Level of Evidence B or C does not imply that the recommendation is weak. Many important clinical questions addressed in the guidelines do not lend themselves to clinical trials. Although randomized trials are unavailable, there may be a very clear clinical consensus that a particular test or therapy is useful or effective.

* Data available from clinical trials or registries about the usefulness/efficacy in different subpopulations, such as sex, age, history of diabetes, history of prior myocardial infarction, history of heart failure, and prior aspirin use.

For comparative effectiveness recommendations (Class I and IIa; Level of Evidence A and B only), studies that support the use of comparator verbs should involve direct comparisons of the treatments or strategies being evaluated.

COmRaIyII/:might be consCidOeRredIII: NomBaeyn/emfiitght be reasoHnaarbmle

COR III: No Benefit

COR III: Harm

is nuostefulness/effectivepnoetsesntisally recoumnkmneonwdne/dunclear/uhnacrmerftualin is noort innodticwaetelldestablischaeudses harm

is not recommended

is not indicated

potentially harmful

causes harm

should not

associated with should not be

associated with

be done

excess morbid- pberdfoornmeed/

excess morbid-

is not useful/ beneficial/

ity/mor tality should not

aobisdtehmnneoeirnfticiusistaeel/rfeudl//

ity/mor tality should not be

effective

be done

eisffneoctivueseful/

pberdfoornmeed/

beneficial/ effective

administered/ other

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2. Clinical Definition

The generally accepted definition of hypertrophic cardiomyopathy (HCM), is a disease state characterized by unexplained left ventricular (LV) hypertrophy associated with nondilated ventricular chambers in the absence of another cardiac or systemic disease that itself would be capable of producing the magnitude of hypertrophy evident in a given patient. Clinically, HCM is usually recognized by maximal LV wall thickness 15 mm, with wall thickness of 13 to 14 mm considered borderline, particularly in the presence of other compelling information (e.g., family history of HCM), based on echocardiography. In terms of LV wall-thickness measurements, the literature has been largely focused on echocardiography, although cardiovascular magnetic resonance (CMR) is now used with increasing frequency in HCM. In the case of children, increased LV wall thickness is defined as wall thickness 2 standard deviations above the mean (z score 2) for age, sex, or body size. However, it should be underscored that in principle, any degree of wall thickness is compatible with the presence of the HCM genetic substrate and that an emerging subgroup within the broad clinical spectrum is composed of family members with disease-causing sarcomere mutations but without evidence of the disease phenotype (i.e., LV hypertrophy).

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