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 (www.cardiosource. org) and the American Heart Association (my.americanheart.org). For copies of this document, please contact Elsevier Inc. Reprint Department, e-mail: [email protected]; 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 [email protected]. 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 2 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. 3 Table 1. Applying ClassificationTableTable 1. of Applying1. Recommendations Applying Classification Classification and Level of ofRecommendations of Evidence and Level of Evidence Recommendations and Level of Evidence SIZE OF TREAT MENSIZET E FFECOF TREAT T MENT E FFECT CLASS I CLASSCLASS IIa I CLASSCLASS IIb IIa CLASSCLASS III IIb No Benefit CLASS III No Benefit Benefit >>> Risk BenefitBenefit >> >>> Risk Risk BenefitBenefit > >> Risk Risk orBenefit CLASS > III Risk Harm or CLASS III Harm Additional studies with AdditionalAdditional studies studies with with broad AdditionalProcedure/ studies with broad Procedure/ Procedure/Treatment Procedure/Treatment Test Treatment Test Treatment focused objectives needed objectivesfocused objectivesneeded; additional needed objectives needed; additional SHOULD be performed/ SHOULD be performed/ COR III: Not No Proven COR III: Not No Proven administered IT administeredIS REASONABLE to per- registryIT IS REASONABLE data would be to helpful per- Noregistry benefit dataHelpful would Benefitbe helpful No benefit Helpful Benefit form procedure/administer Procedure/Treatmentform procedure/administer CORProcedure/Treatment III: Excess Cost Harmful COR III: Excess Cost Harmful treatment MAYtreatment BE CONSIDERED HarmMAY BE w/oCONS BenefitIDERED to Patients Harm w/o Benefit to Patients or Harmful or Harmful T T LEVEL A n RecommendationLEVEL A that n Recommendationn Recommendation in thatfavor n nRecommendation’s Recommendation in favor n nRecommendation Recommendation’s that n Recommendation that FFEC FFEC procedure or treatment of proceduretreatment or proceduretreatment usefulness/efficacyof treatment or procedure less procedureusefulness/efficacy or treatment less is procedure or treatment is E Multiple populations E Multiple populations is useful/effective beingis useful/effective useful/effective wellbeing established useful/effective notwell useful/effective established and may T not useful/effective and may evaluated* T evaluated* n Sufficient evidence from n Somen Sufficient conflicting evidence evidence from n nGreater Some conflicting evidence ben harmful Greater conflicting be harmful MEN Data derived from multiple MEN Data derived from multiple multiple randomized trials frommultiple multiple randomized randomized trials n n T evidencefrom multiple from multiple randomized T evidenceSufficient from evidence multiple from Sufficient evidence from randomized clinical trials or meta-analysesrandomized clinical trials trialsor meta-analyses or meta-analyses randomizedtrials or meta-analyses trials or multiplerandomized randomized trials or trials or multiple randomized trials or or meta-analyses or meta-analyses meta-analyses meta-analysesmeta-analyses meta-analyses LEVEL B n RecommendationLEVEL B that n Recommendationn Recommendation in thatfavor n nRecommendation’s Recommendation in favor n nRecommendation Recommendation’s that n Recommendation that procedure or treatment of proceduretreatment or proceduretreatment usefulness/efficacyof treatment or procedure less procedureusefulness/efficacy or treatment less is procedure or treatment is ION) OF TREA Limited populations ION) OF TREA Limited populations S S is useful/effective beingis useful/effective useful/effective wellbeing established useful/effective notwell useful/effective established and may not useful/effective and may evaluated* evaluated* n Evidence from single n Somen Evidence conflicting from single n nGreater Some conflicting ben harmful Greater conflicting be harmful Data derived from a Data derived from a randomized trial or evidencerandomized from trialsingle or evidenceevidence from from single single n evidenceEvidence fromfrom singlesingle n Evidence from single single randomized trial single randomized trial Y (PRECI randomized trial or Y (PRECI nonrandomized studies nonrandomized studies randomizedrandomized trial trial or or randomizedrandomized trial trial or or randomized trial or T or nonrandomized studies T or nonrandomized studies nonrandomized studies nonrandomizednonrandomized studies studies nonrandomizednonrandomized studies studies nonrandomized studies AIN AIN T T LEVEL C n RecommendationLEVEL C that n Recommendationn Recommendation in thatfavor n nRecommendation’s Recommendation in favor n Recommendation’s ER ER n Recommendation that n Recommendation that C C procedure or treatment is of proceduretreatment or proceduretreatment is usefulness/efficacyof treatment or procedure less usefulness/efficacy less Very limited populations Very limited populations procedure or treatment is procedure or treatment is useful/effective beinguseful/effective useful/effective wellbeing established useful/effective well established evaluated* evaluated* not useful/effective and may not useful/effective and may E OF E OF n Only expert opinion, case n Onlyn Only diverging expert opinion, expert case n nOnly Only diverging diverging expert expert n Only diverging expert T Only consensus opinion T Only consensus opinion be harmful be harmful studies, or standard of care opinion,studies, case or standard studies, of care opinion,opinion, case case studies, studies, or opinion, case studies, or of experts, case studies, of experts, case studies, n Only expert opinion, case n Only expert opinion, case IMA IMA or standard of care standardor standard of care of care standard of care or standard of care or standard of care studies, or standard of care studies, or standard of care EST EST Suggested phrases for shouldSuggested phrases for is reasonableshould may/mightis reasonable be considered CORmay/might III: be consideredCOR III: COR III: COR III: writing recommendations is recommendedwriting recommendations canis be recommended useful/effective/beneficial may/mightcan be useful/effective/beneficial be reasonable Nomay/might Benefit be reasonableHarm No Benefit Harm