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Circulation. 2008;118:e523-e661
Published online before print September 26, 2008, doi: 10.1161/CIRCULATIONAHA.108.190748
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(Circulation. 2008;118:e523-e661.)
© 2008 American Heart Association, Inc.

Practice Guideline

2008 Focused Update Incorporated Into the 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): Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

2006 WRITING COMMITTEE MEMBERS; Robert O. Bonow, MD, MACC, FAHA, Chair; Blase A. Carabello, MD, FACC, FAHA; Kanu Chatterjee, MB, FACC; Antonio C. de Leon, Jr, MD, FACC, FAHA; David P. Faxon, MD, FACC, FAHA; Michael D. Freed, MD, FACC, FAHA; William H. Gaasch, MD, FACC, FAHA; Bruce W. Lytle, MD, FACC, FAHA; Rick A. Nishimura, MD, FACC, FAHA; Patrick T. O'Gara, MD, FACC, FAHA; Robert A. O'Rourke, MD, MACC, FAHA; Catherine M. Otto, MD, FACC, FAHA; Pravin M. Shah, MD, MACC, FAHA; Jack S. Shanewise, MD*


Key Words: ACC/AHA practice guideline • valvular heart disease • heart valves • cardiac murmur • valve lesion • thoracic surgery


*    TABLE OF CONTENTS
up arrowTop
 *TABLE OF CONTENTS
down arrowPreamble (Updated)
 down arrow1. Introduction
 down arrow2. General Principles
 down arrow3. Specific Valve Lesions
 down arrow4. Evaluation and Management...
 down arrow5. Management of Valvular...
 down arrow6. Management of Congenital...
 down arrow7. Surgical Considerations
 down arrow8. Intraoperative Assessment
 down arrow9. Management of Patients...
 down arrow10. Evaluation and Treatment...
 down arrowStaff. - 2006 Guideline
 down arrowStaff. - 2008 Focused...
 down arrowReferences
 

Preamble (updated) e526
  1.    Introduction e527
          1.1. Evidence Review (UPDATED) e527
          1.2. Scope of the Document (UPDATED) e528
          1.3. Review and Approval (NEW) e529

  2.    General Principles e530
          2.1. Evaluation of the Patient With a Cardiac Murmur e530
             2.1.1. Introduction (UPDATED) e530
             2.1.2. Classification of Murmurs e530
                2.1.2.1. Dynamic Cardiac Auscultation e531
                2.1.2.2. Other Physical Findings e531
                2.1.2.3. Associated Symptoms e532

             2.1.3. Electrocardiography and Chest Roentgenography e533
             2.1.4. Echocardiography e533
             2.1.5. Cardiac Catheterization e533
             2.1.6. Exercise Testing e533
             2.1.7. Approach to the Patient e534

          2.2. Valve Disease Severity Table e535
          2.3. Endocarditis and Rheumatic Fever Prophylaxis (UPDATED) e535
             2.3.1. Endocarditis Prophylaxis (UPDATED) e535
                Table 5 (DELETED)
                Table 6 (UPDATED)
                Table 7 (UPDATED)
                Table 8 (DELETED)

             2.3.2. Rheumatic Fever Prophylaxis e538
                2.3.2.1. General Considerations e538
                2.3.2.2. Primary Prevention e538
                2.3.2.3. Secondary Prevention e538



  3.    Specific Valve Lesions e539
          3.1. Aortic Stenosis e539
             3.1.1. Introduction e539
                3.1.1.1. Grading the Degree of Stenosis e539

             3.1.2. Pathophysiology e540
             3.1.3. Natural History e540
             3.1.4. Management of the Asymptomatic Patient e541
                3.1.4.1. Echocardiography (Imaging, Spectral, and Color Doppler) in Aortic Stenosis e542
                3.1.4.2. Exercise Testing e542
                3.1.4.3. Serial Evaluations e543
                3.1.4.4. Medical Therapy (UPDATED) e543
                3.1.4.5. Physical Activity and Exercise e543

             3.1.5. Indications for Cardiac Catheterization e543
             3.1.6. Low-Flow/Low-Gradient Aortic Stenosis e544
             3.1.7. Indications for Aortic Valve Replacement e544
                3.1.7.1. Symptomatic Patients e545
                3.1.7.2. Asymptomatic Patients e546
                3.1.7.3. Patients Undergoing Coronary Artery Bypass or Other Cardiac Surgery e546

             3.1.8. Aortic Balloon Valvotomy e546
             3.1.9. Medical Therapy for the Inoperable Patient e547
             3.1.10. Evaluation After Aortic Valve Replacement e547
             3.1.11. Special Considerations in the Elderly e547

          3.2. Aortic Regurgitation e547
             3.2.1. Etiology e547
             3.2.2. Acute Aortic Regurgitation e548
                3.2.2.1. Pathophysiology e548
                3.2.2.2. Diagnosis e548
                3.2.2.3. Treatment e548

             3.2.3. Chronic Aortic Regurgitation e548
                3.2.3.1. Pathophysiology e548
                3.2.3.2. Natural History e549
                   3.2.3.2.1. Asymptomatic Patients With Normal Left Ventricular Function e549
                   3.2.3.2.2. Asymptomatic Patients With Depressed Systolic Function e550
                   3.2.3.2.3. Symptomatic Patients e551

                3.2.3.3. Diagnosis and Initial Evaluation e552
                3.2.3.4. Medical Therapy e553
                3.2.3.5. Physical Activity and Exercise e554
                3.2.3.6. Serial Testing e555
                3.2.3.7. Indications for Cardiac Catheterization e555
                3.2.3.8. Indications for Aortic Valve Replacement or Aortic Valve Repair e556
                   3.2.3.8.1. Symptomatic Patients With Normal Left Ventricular Systolic Function e556
                   3.2.3.8.2. Symptomatic Patients With Left Ventricular Dysfunction e557
                   3.2.3.8.3. Asymptomatic Patients e557


             3.2.4. Concomitant Aortic Root Disease e558
             3.2.5. Evaluation of Patients After Aortic Valve Replacement e558
             3.2.6. Special Considerations in the Elderly e559

          3.3. Bicuspid Aortic Valve With Dilated Ascending Aorta e559
          3.4. Mitral Stenosis e560
             3.4.1. Pathophysiology and Natural History e560
             3.4.2. Indications for Echocardiography in Mitral Stenosis e561
             3.4.3. Medical Therapy e563
                3.4.3.1. Medical Therapy: General (UPDATED) e563
                3.4.3.2. Medical Therapy: Atrial Fibrillation e563
                3.4.3.3. Medical Therapy: Prevention of Systemic Embolization e564

             3.4.4. Recommendations Regarding Physical Activity and Exercise e565
             3.4.5. Serial Testing e565
             3.4.6. Evaluation of the Symptomatic Patient e565
             3.4.7. Indications for Invasive Hemodynamic Evaluation e565
             3.4.8. Indications for Percutaneous Mitral Balloon Valvotomy e568
             3.4.9. Indications for Surgery for Mitral Stenosis e570
             3.4.10. Management of Patients After Valvotomy or Commissurotomy e572
             3.4.11. Special Considerations e572
                3.4.11.1 Pregnant Patients e572
                3.4.11.2. Older Patients e572


          3.5. Mitral Valve Prolapse e573
             3.5.1. Pathophysiology and Natural History e573
             3.5.2. Evaluation and Management of the Asymptomatic Patient (UPDATED) e574
             3.5.3. Evaluation and Management of the Symptomatic Patient (UPDATED) e574
             3.5.4. Surgical Considerations e576

          3.6. Mitral Regurgitation e576
             3.6.1. Etiology e576
             3.6.2. Acute Severe Mitral Regurgitation e576
                3.6.2.1. Pathophysiology e576
                3.6.2.2. Diagnosis e576
                3.6.2.3. Medical Therapy e576

             3.6.3. Chronic Asymptomatic Mitral Regurgitation e577
                3.6.3.1. Pathophysiology and Natural History e577
                3.6.3.2. Diagnosis e577
                3.6.3.3. Indications for Transthoracic Echocardiography e577
                3.6.3.4. Indications for Transesophageal Echocardiography e578
                3.6.3.5. Serial Testing e578
                3.6.3.6. Guidelines for Physical Activity and Exercise e579
                3.6.3.7. Medical Therapy e579
                3.6.3.8. Indications for Cardiac Catheterization e579

             3.6.4. Indications for Surgery e579
                3.6.4.1. Types of Surgery e579
                3.6.4.2. Indications for Mitral Valve Operation e580
                   3.6.4.2.1. Symptomatic Patients With Normal Left Ventricular Function e581
                   3.6.4.2.2. Asymptomatic or Symptomatic Patients With Left Ventricular Dysfunction e581
                   3.6.4.2.3. Asymptomatic Patients With Normal Left Ventricular Function e581
                   3.6.4.2.4. Atrial Fibrillation e582


             3.6.5. Ischemic Mitral Regurgitation e583
             3.6.6. Evaluation of Patients After Mitral Valve Replacement or Repair e584
             3.6.7. Special Considerations in the Elderly e584

          3.7. Multiple Valve Disease e584
             3.7.1. Introduction e584
             3.7.2. Mixed Single Valve Disease e584
                3.7.2.1. Pathophysiology e584
                3.7.2.2. Diagnosis e584
                   3.7.2.2.1. Two-Dimensional and Doppler Echocardiographic Studies e584
                   3.7.2.2.2. Cardiac Catheterization e585

                3.7.2.3. Management e585

             3.7.3. Combined Mitral Stenosis and Aortic Regurgitation e585
                3.7.3.1. Pathophysiology e585
                3.7.3.2. Management e585

             3.7.4. Combined Mitral Stenosis and Tricuspid Regurgitation e585
                3.7.4.1. Pathophysiology e585
                3.7.4.2. Diagnosis e585
                3.7.4.3. Management e585

             3.7.5. Combined Mitral Regurgitation and Aortic Regurgitation e586
                3.7.5.1. Pathophysiology e586
                3.7.5.2. Diagnosis and Therapy e586

             3.7.6. Combined Mitral Stenosis and Aortic Stenosis e586
                3.7.6.1. Pathophysiology e586
                3.7.6.2. Diagnosis and Therapy e586

             3.7.7. Combined Aortic Stenosis and Mitral Regurgitation e586
                3.7.7.1. Pathophysiology e586
                3.7.7.2. Diagnosis and Therapy e586


          3.8. Tricuspid Valve Disease e586
             3.8.1. Pathophysiology e586
             3.8.2. Diagnosis e587
             3.8.3. Management e587

          3.9. Drug-Related Valvular Heart Disease e588
          3.10. Radiation Heart Disease e588

  4.    Evaluation and Management of Infective Endocarditis e589
          4.1. Antimicrobial Therapy e589
          4.2. Culture-Negative Endocarditis e589
          4.3. Endocarditis in HIV-Seropositive Patients e590
          4.4. Indications for Echocardiography in Suspected or Known Endocarditis e591
             4.4.1. Transthoracic Echocardiography in Endocarditis e592
             4.4.2. Transesophageal Echocardiography in Endocarditis e592

          4.5. Outpatient Treatment e593
          4.6. Indications for Surgery in Patients With Acute Infective Endocarditis e593
             4.6.1. Surgery for Native Valve Endocarditis e595
             4.6.2. Surgery for Prosthetic Valve Endocarditis e596


  5.    Management of Valvular Disease in Pregnancy e596
          5.1. Physiological Changes of Pregnancy e596
          5.2. Physical Examination e598
          5.3. Echocardiography e598
          5.4. General Management Guidelines e598
          5.5. Specific Lesions e599
             5.5.1. Mitral Stenosis e599
             5.5.2. Mitral Regurgitation e599
             5.5.3. Aortic Stenosis e599
             5.5.4. Aortic Regurgitation e601
             5.5.5. Pulmonic Stenosis e601
             5.5.6. Tricuspid Valve Disease e601
             5.5.7. Marfan Syndrome e601

          5.6. Endocarditis Prophylaxis (UPDATED) e601
          5.7. Cardiac Valve Surgery e601
          5.8. Anticoagulation During Pregnancy e602
             5.8.1. Warfarin e602
             5.8.2. Unfractionated Heparin e602
             5.8.3. Low-Molecular-Weight Heparins e602
             5.8.4. Selection of Anticoagulation Regimen in Pregnant Patients With Mechanical Prosthetic Valves e602

          5.9. Selection of Valve Prostheses in Young Women e604

  6.    Management of Congenital Valvular Heart Disease in Adolescents and Young Adults (UPDATED) e604
          6.1. Aortic Stenosis e604
             6.1.1. Pathophysiology e604
             6.1.2. Evaluation of Asymptomatic Adolescents or Young Adults With Aortic Stenosis e604
             6.1.3. Indications for Aortic Balloon Valvotomy in Adolescents and Young Adults e605

          6.2. Aortic Regurgitation e606
          6.3. Mitral Regurgitation e607
          6.4. Mitral Stenosis e608
          6.5. Tricuspid Valve Disease e608
             6.5.1. Pathophysiology e608
             6.5.2. Evaluation of Tricuspid Valve Disease in Adolescents and Young Adults e609
             6.5.3. Indications for Intervention in Tricuspid Regurgitation e609

          6.6. Pulmonic Stenosis e610
             6.6.1. Pathophysiology e610
             6.6.2. Evaluation of Pulmonic Stenosis in Adolescents and Young Adults e610
             6.6.3. Indications for Balloon Valvotomy in Pulmonic Stenosis (UPDATED) e610

          6.7. Pulmonary Regurgitation e611

  7.    Surgical Considerations e612
          7.1. American Association for Thoracic Surgery/Society of Thoracic Surgeons Guidelines for Clinical Reporting of Heart Valve Complications e612
          7.2. Aortic Valve Surgery e614
             7.2.1. Risks and Strategies in Aortic Valve Surgery e614
             7.2.2. Mechanical Aortic Valve Prostheses e614
                7.2.2.1. Antithrombotic Therapy for Patients With Aortic Mechanical Heart Valves e614

             7.2.3. Stented and Nonstented Heterografts e614
                7.2.3.1. Aortic Valve Replacement With Stented Heterografts e614
                7.2.3.2. Aortic Valve Replacement With Stentless Heterografts e616

             7.2.4. Aortic Valve Homografts e616
             7.2.5. Pulmonic Valve Autotransplantation e616
             7.2.6. Aortic Valve Repair e617
             7.2.7. Left Ventricle–to–Descending Aorta Shunt e617
             7.2.8. Comparative Trials and Selection of Aortic Valve Prostheses e617
             7.2.9. Major Criteria for Aortic Valve Selection e618

          7.3. Mitral Valve Surgery e618
             7.3.1. Mitral Valve Repair e619
                7.3.1.1. Myxomatous Mitral Valve e619
                7.3.1.2. Rheumatic Heart Disease e619
                7.3.1.3. Ischemic Mitral Valve Disease e619
                7.3.1.4. Mitral Valve Endocarditis e620

             7.3.2. Mitral Valve Prostheses (Mechanical or Bioprostheses) e620
                7.3.2.1. Selection of a Mitral Valve Prosthesis e620
                7.3.2.2. Choice of Mitral Valve Operation e620


          7.4. Tricuspid Valve Surgery e621
          7.5. Valve Selection for Women of Childbearing Age e621

  8.    Intraoperative Assessment e621
          8.1. Specific Valve Lesions e622
             8.1.1. Aortic Stenosis e622
             8.1.2. Aortic Regurgitation e622
             8.1.3. Mitral Stenosis e622
             8.1.4. Mitral Regurgitation e623
             8.1.5. Tricuspid Regurgitation e623
             8.1.6. Tricuspid Stenosis e623
             8.1.7. Pulmonic Valve Lesions e623

          8.2. Specific Clinical Scenarios e623
             8.2.1. Previously Undetected Aortic Stenosis During CABG e623
             8.2.2. Previously Undetected Mitral Regurgitation During CABG e623


  9.    Management of Patients With Prosthetic Heart Valves e624
          9.1. Antibiotic Prophylaxis e624
             9.1.1. Infective Endocarditis e624
             9.1.2. Recurrence of Rheumatic Carditis e624

          9.2. Antithrombotic Therapy e624
             9.2.1. Mechanical Valves e625
             9.2.2. Biological Valves e625
             9.2.3. Embolic Events During Adequate Antithrombotic Therapy e626
             9.2.4. Excessive Anticoagulation e626
             9.2.5. Bridging Therapy in Patients With Mechanical Valves Who Require Interruption of Warfarin Therapy for Noncardiac Surgery, Invasive Procedures, or Dental Care e626
             9.2.6. Antithrombotic Therapy in Patients Who Need Cardiac Catheterization/Angiography e627
             9.2.7. Thrombosis of Prosthetic Heart Valves e627

          9.3. Follow-Up Visits e628
             9.3.1. First Outpatient Postoperative Visit e628
             9.3.2. Follow-Up Visits in Patients Without Complications e629
             9.3.3. Follow-Up Visits in Patients With Complications e629

          9.4. Reoperation to Replace a Prosthetic Valve e629

  10.    Evaluation and Treatment of Coronary Artery Disease in Patients With Valvular Heart Disease e630
          10.1. Probability of Coronary Artery Disease in Patients With Valvular Heart Disease e630
          10.2. Diagnosis of Coronary Artery Disease e630
          10.3. Treatment of Coronary Artery Disease at the Time of Aortic Valve Replacement e631
          10.4. Aortic Valve Replacement in Patients Undergoing Coronary Artery Bypass Surgery e631
          10.5. Management of Concomitant MV Disease and Coronary Artery Disease e632


References e633
Appendix 1 e656
Appendix 2 e657
Appendix 3 e659
Appendix 4 (NEW) e659
Appendix 5 (NEW) e660


*    Preamble (Updated)
up arrowTop
 up arrowTABLE OF CONTENTS
 *Preamble (Updated)
down arrow1. Introduction
 down arrow2. General Principles
 down arrow3. Specific Valve Lesions
 down arrow4. Evaluation and Management...
 down arrow5. Management of Valvular...
 down arrow6. Management of Congenital...
 down arrow7. Surgical Considerations
 down arrow8. Intraoperative Assessment
 down arrow9. Management of Patients...
 down arrow10. Evaluation and Treatment...
 down arrowStaff. - 2006 Guideline
 down arrowStaff. - 2008 Focused...
 down arrowReferences
 
It is important that the medical profession play a significant role in critically evaluating the use of diagnostic procedures and therapies as they are introduced in the detection, management, or prevention of disease states. Rigorous and expert analysis of the available data documenting the absolute and relative benefits and risks of those procedures and therapies can produce helpful guidelines that improve the effectiveness of care, optimize patient outcomes, and favorably affect the overall cost of care by focusing resources on the most effective strategies.

The American College of Cardiology (ACC) and the American Heart Association (AHA) have jointly engaged in the production of such guidelines in the area of cardiovascular disease since 1980. This effort is directed by the ACC/AHA Task Force on Practice Guidelines, whose charge is to develop, update, or revise practice guidelines for important cardiovascular diseases and procedures. Writing committees are charged with the task of performing an assessment of the evidence and acting as an independent group of authors to develop and update written recommendations for clinical practice.

Experts in the subject under consideration are selected from both organizations to examine subject-specific data and write guidelines. The process includes additional representatives from other medical practitioner and specialty groups where appropriate. Writing committees are specifically charged to perform a formal literature review, weigh the strength of evidence for or against a particular treatment or procedure, and include estimates of expected health outcomes where data exist. Patient-specific modifiers, comorbidities, and issues of patient preference that may influence the choice of particular tests or therapies are considered, as well as frequency of follow-up. When available, information from studies on cost will be considered; however, review of data on efficacy and clinical outcomes will be the primary basis for preparing recommendations in these guidelines.

The ACC/AHA Task Force on Practice Guidelines makes every effort to avoid any actual, potential, or perceived conflicts of interest that may arise as a result of an outside relationship or personal interest of a member of the writing committee. Specifically, all members of the writing committee and peer reviewers of the document are asked to provide disclosure statements of all such relationships that may be perceived as real or potential conflicts of interest. Writing committee members are also strongly encouraged to declare a previous relationship with industry that may be perceived as relevant to guideline development. If a writing committee member develops a new relationship with industry during his or her tenure, he or she is required to notify guideline staff in writing. The continued participation of the writing committee member will be reviewed. These statements are reviewed by the parent task force, reported orally to all members of the writing panel at each meeting, and updated and reviewed by the writing committee as changes occur. Please refer to the methodology manual for the ACC/AHA guideline writing committees for further description and the relationships with industry policy.1067 See Appendix 1 for a list of writing committee member relationships with industry and Appendix 2 for a listing of peer reviewer relationships with industry that are pertinent to this guideline.

These practice guidelines are intended to assist healthcare providers in clinical decision making by describing a range of generally acceptable approaches for the diagnosis, management, and prevention of specific diseases or conditions. See Appendix 3 for a list of abbreviated terms used in this guideline. These guidelines attempt to define practices that meet the needs of most patients in most circumstances. These guideline recommendations reflect a consensus of expert opinion after a thorough review of the available, current scientific evidence and are intended to improve patient care. If these guidelines are used as the basis for regulatory/payer decisions, the ultimate goal is quality of care and serving the patient’s best interests. The ultimate judgment regarding care of a particular patient must be made by the healthcare provider and patient in light of all of the circumstances presented by that patient. There are circumstances in which deviations from these guidelines are appropriate.

The current document is a republication of the "ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease,"1068 revised to incorporate individual recommendations from a 2008 focused update,1069 which spotlights the 2007 AHA Guidelines for Infective Endocarditis Prophylaxis. For easy reference, this online-only version denotes sections that have been updated. All members of the 2006 Valvular Heart Disease Writing Committee were invited to participate in the writing group; those who agreed were required to disclose all relationships with industry relevant to the data under consideration,1067 as were all peer reviewers of the document. (See Appendixes 4 and 5 for a listing of relationships with industry for the 2008 Focused Update Writing Group and peer reviewers, respectively.) Each recommendation required a confidential vote by the writing group members before and after external review of the document. Any writing group member with a significant (greater than $10 000) relationship with industry relevant to the recommendation was recused from voting on that recommendation.

Guidelines are reviewed annually by the ACC/AHA Task Force on Practice Guidelines and are considered current unless they are updated or sunsetted and withdrawn from distribution.

Sidney C. Smith, Jr., MD, FACC, FAHA

Chair, ACC/AHA Task Force on Practice Guidelines


*    1. Introduction
up arrowTop
 up arrowTABLE OF CONTENTS
 up arrowPreamble (Updated)
 *1. Introduction
down arrow2. General Principles
 down arrow3. Specific Valve Lesions
 down arrow4. Evaluation and Management...
 down arrow5. Management of Valvular...
 down arrow6. Management of Congenital...
 down arrow7. Surgical Considerations
 down arrow8. Intraoperative Assessment
 down arrow9. Management of Patients...
 down arrow10. Evaluation and Treatment...
 down arrowStaff. - 2006 Guideline
 down arrowStaff. - 2008 Focused...
 down arrowReferences
 
1.1. Evidence Review (UPDATED)
The ACC and the AHA have long been involved in the joint development of practice guidelines designed to assist healthcare providers in the management of selected cardiovascular disorders or the selection of certain cardiovascular procedures. The determination of the disorders or procedures to develop guidelines is based on several factors, including importance to healthcare providers and whether there are sufficient data from which to derive accepted guidelines. One important category of cardiac disorders that affect a large number of patients who require diagnostic procedures and decisions regarding long-term management is valvular heart disease.

During the past 2 decades, major advances have occurred in diagnostic techniques, the understanding of natural history, and interventional cardiology and surgical procedures for patients with valvular heart disease. These advances have resulted in enhanced diagnosis, more scientific selection of patients for surgery or catheter-based intervention versus medical management, and increased survival of patients with these disorders. The information base from which to make clinical management decisions has greatly expanded in recent years, yet in many situations, management issues remain controversial or uncertain. Unlike many other forms of cardiovascular disease, there is a scarcity of large-scale multicenter trials addressing the diagnosis and treatment of patients with valvular disease from which to derive definitive conclusions, and the information available in the literature represents primarily the experiences reported by single institutions in relatively small numbers of patients.

The 1998 Committee on Management of Patients With Valvular Heart Disease reviewed and compiled this information base and made recommendations for diagnostic testing, treatment, and physical activity. For topics for which there was an absence of multiple randomized, controlled trials, the preferred basis for medical decision making in clinical practice (evidence-based medicine), the committee’s recommendations were based on data derived from single randomized trials or nonrandomized studies or were based on a consensus opinion of experts. The 2006 writing committee was charged with revising the guidelines published in 1998. The committee reviewed pertinent publications, including abstracts, through a computerized search of the English literature since 1998 and performed a manual search of final articles. Special attention was devoted to identification of randomized trials published since the original document. A complete listing of all publications covering the treatment of valvular heart disease is beyond the scope of this document; the document includes those reports that the committee believes represent the most comprehensive or convincing data that are necessary to support its conclusions. However, evidence tables were updated to reflect major advances over this time period. Inaccuracies or inconsistencies present in the original publication were identified and corrected when possible. Recommendations provided in this document are based primarily on published data. Because randomized trials are unavailable in many facets of valvular heart disease treatment, observational studies, and, in some areas, expert opinions form the basis for recommendations that are offered.

All of the recommendations in this guideline revision were converted from the tabular format used in the 1998 guideline to a listing of recommendations that has been written in full sentences to express a complete thought, such that a recommendation, even if separated and presented apart from the rest of the document, would still convey the full intent of the recommendation. It is hoped that this will increase the readers’ comprehension of the guidelines. Also, the level of evidence, either A, B, or C, for each recommendation is now provided.

Classification of recommendations and level of evidence are expressed in the ACC/AHA format as follows:

In addition, the weight of evidence in support of the recommendation is listed as follows:

The schema for classification of recommendations and level of evidence is summarized in Fig. 1, which also illustrates how the grading system provides an estimate of the size of the treatment effect and an estimate of the certainty of the treatment effect.


Figure 113968
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  		Figure 1. Applying classification of recommendations and level of evidence.

*Data available from clinical trials or registries about the usefulness/efficacy in different subpopulations, such as gender, age, history of diabetes, history of prior myocardial infarction, history of heart failure, and prior aspirin use. 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. Even though randomized trials are not available, there may be a very clear clinical consensus that a particular test or therapy is useful or effective. {dagger}In 2003 the ACC/AHA Task Force on Practice Guidelines recently provided a list of suggested phrases to use when writing recommendations. All recommendations in this guideline have been written in full sentences that express a complete thought, such that a recommendation, even if separated and presented apart from the rest of the document (including headings above sets of recommendations), would still convey the full intent of the recommendation. It is hoped that this will increase readers’ comprehension of the guidelines and will allow queries at the individual recommendation level.

Writing committee membership consisted of cardiovascular disease specialists and representatives of the cardiac surgery and cardiac anesthesiology fields; both the academic and private practice sectors were represented. The Society of Cardiovascular Anesthesiologists assigned an official representative to the writing committee.

1.2. Scope of the Document (UPDATED)
The guidelines attempt to deal with general issues of treatment of patients with heart valve disorders, such as evaluation of patients with heart murmurs, prevention and treatment of endocarditis, management of valve disease in pregnancy, and treatment of patients with concomitant coronary artery disease (CAD), as well as more specialized issues that pertain to specific valve lesions. The guidelines focus primarily on valvular heart disease in the adult, with a separate section dealing with specific recommendations for valve disorders in adolescents and young adults. The diagnosis and management of infants and young children with congenital valvular abnormalities are significantly different from those of the adolescent or adult and are beyond the scope of these guidelines.

This task force report overlaps with several previously published ACC/AHA guidelines about cardiac imaging and diagnostic testing, including the guidelines for the clinical use of cardiac radionuclide imaging,1 the clinical application of echocardiography,2 exercise testing,3 and percutaneous coronary intervention.4 Although these guidelines are not intended to include detailed information covered in previous guidelines on the use of imaging and diagnostic testing, an essential component of this report is the discussion of indications for these tests in the evaluation and treatment of patients with valvular heart disease.

The committee emphasizes the fact that many factors ultimately determine the most appropriate treatment of individual patients with valvular heart disease within a given community. These include the availability of diagnostic equipment and expert diagnosticians, the expertise of interventional cardiologists and surgeons, and notably, the wishes of well-informed patients. Therefore, deviation from these guidelines may be appropriate in some circumstances. These guidelines are written with the assumption that a diagnostic test can be performed and interpreted with skill levels consistent with previously reported ACC training and competency statements and ACC/AHA guidelines, that interventional cardiological and surgical procedures can be performed by highly trained practitioners within acceptable safety standards, and that the resources necessary to perform these diagnostic procedures and provide this care are readily available. This is not true in all geographic areas, which further underscores the committee’s position that its recommendations are guidelines and not rigid requirements.

1.3. Review and Approval (NEW)
The 2006 document1068 was reviewed by 2 official reviewers nominated by the ACC; 2 official reviewers nominated by the AHA; 1 official reviewer from the ACC/AHA Task Force on Practice Guidelines; reviewers nominated by the Society of Cardiovascular Anesthesiologists, the Society for Cardiovascular Angiography and Interventions, and the Society of Thoracic Surgeons (STS); and individual content reviewers, including members of the ACCF Cardiac Catheterization and Intervention Committee, ACCF Cardiovascular Imaging Committee, ACCF Cardiovascular Surgery Committee, AHA Endocarditis Committee, AHA Cardiac Clinical Imaging Committee, AHA Cardiovascular Intervention and Imaging Committee, and AHA Cerebrovascular Imaging and Intervention Committee.

As mentioned previously, this document also incorporates a 2008 focused update of the "ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease,"1069 which spotlights the 2007 AHA Guidelines for Infective Endocarditis Prophylaxis.1070 Only recommendations related to infective endocarditis have been revised. This document was reviewed by 2 external reviewers nominated by the ACC and 2 external reviewers nominated by the AHA, as well as 3 reviewers from the ACCF Congenital Heart Disease and Pediatric Committee, 2 reviewers from the ACCF Cardiovascular Surgery Committee, 5 reviewers from the AHA Heart Failure and Transplant Committee, and 3 reviewers from the Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee. All information about reviewers’ relationships with industry was collected and distributed to the writing committee and is published in this document (see Appendix 5 for details). This document was approved for publication by the governing bodies of the ACCF and the AHA in May 2008 and endorsed by the Society of Cardiovascular Anesthesiologists, the Society for Cardiovascular Angiography and Interventions, and the Society of Thoracic Surgeons.


*    2. General Principles
up arrowTop
 up arrowTABLE OF CONTENTS
 up arrowPreamble (Updated)
 up arrow1. Introduction
 *2. General Principles
down arrow3. Specific Valve Lesions
 down arrow4. Evaluation and Management...
 down arrow5. Management of Valvular...
 down arrow6. Management of Congenital...
 down arrow7. Surgical Considerations
 down arrow8. Intraoperative Assessment
 down arrow9. Management of Patients...
 down arrow10. Evaluation and Treatment...
 down arrowStaff. - 2006 Guideline
 down arrowStaff. - 2008 Focused...
 down arrowReferences
 
2.1. Evaluation of the Patient With a Cardiac Murmur
2.1.1. Introduction (UPDATED)
Cardiac auscultation remains the most widely used method of screening for valvular heart disease (VHD). The production of murmurs is due to 3 main factors:

Often, more than 1 of these factors is operative.5–7

A heart murmur may have no pathological significance or may be an important clue to the presence of valvular, congenital, or other structural abnormalities of the heart.8 Most systolic heart murmurs do not signify cardiac disease, and many are related to physiological increases in blood flow velocity.9 In other instances, a heart murmur may be an important clue to the diagnosis of undetected cardiac disease (e.g., valvular aortic stenosis [AS]) that may be important even when asymptomatic or that may define the reason for cardiac symptoms. In these situations, various noninvasive or invasive cardiac tests may be necessary to establish a firm diagnosis and form the basis for rational treatment of an underlying disorder. Echocardiography is particularly useful in this regard, as discussed in the "ACC/AHA/ASE 2003 Guidelines for the Clinical Application of Echocardiography."2 Diastolic murmurs virtually always represent pathological conditions and require further cardiac evaluation, as do most continuous murmurs. Continuous "innocent" murmurs include venous hums and mammary souffles.

The traditional auscultation method of assessing cardiac murmurs has been based on their timing in the cardiac cycle, configuration, location and radiation, pitch, intensity (grades 1 through 6), and duration.5–9 The configuration of a murmur may be crescendo, decrescendo, crescendo-decrescendo (diamond-shaped), or plateau. The precise times of onset and cessation of a murmur associated with cardiac pathology depend on the period of time in the cardiac cycle in which a physiologically important pressure difference between 2 chambers occurs.5–9 A classification of cardiac murmurs is listed in Table 1.


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  		Table 1. Classification of Cardiac Murmurs

2.1.2. Classification of Murmurs
Holosystolic (pansystolic) murmurs are generated when there is flow between chambers that have widely different pressures throughout systole, such as the left ventricle and either the left atrium or right ventricle. With an abnormal regurgitant orifice, the pressure gradient and regurgitant jet begin early in contraction and last until relaxation is almost complete.

Midsystolic (systolic ejection) murmurs, often crescendo-decrescendo in configuration, occur when blood is ejected across the aortic or pulmonic outflow tracts. The murmurs start shortly after S1, when the ventricular pressure rises sufficiently to open the semilunar valve. As ejection increases, the murmur is augmented, and as ejection declines, it diminishes.

In the presence of normal semilunar valves, this murmur may be caused by an increased flow rate such as that which occurs with elevated cardiac output (e.g., pregnancy, thyrotoxicosis, anemia, and arteriovenous fistula), ejection of blood into a dilated vessel beyond the valve, or increased transmission of sound through a thin chest wall. Most innocent murmurs that occur in children and young adults are midsystolic and originate either from the aortic or pulmonic outflow tracts. Valvular, supravalvular, or subvalvular obstruction (stenosis) of either ventricle may also cause a midsystolic murmur, the intensity of which depends in part on the velocity of blood flow across the narrowed area. Midsystolic murmurs also occur in certain patients with functional mitral regurgitation (MR) or, less frequently, tricuspid regurgitation (TR). Echocardiography is often necessary to separate a prominent and exaggerated (grade 3) benign midsystolic murmur from one due to valvular AS.

Early systolic murmurs are less common; they begin with the first sound and end in midsystole. An early systolic murmur is often due to TR that occurs in the absence of pulmonary hypertension, but it also occurs in patients with acute MR. In large ventricular septal defects with pulmonary hypertension and small muscular ventricular septal defects, the shunting at the end of systole may be insignificant, with the murmur limited to early and midsystole.

Late systolic murmurs are soft or moderately loud, high-pitched murmurs at the left ventricular (LV) apex that start well after ejection and end before or at S2. They are often due to apical tethering and malcoaptation of the mitral leaflets due to anatomic and functional changes of the annulus and ventricle. Late systolic murmurs in patients with midsystolic clicks result from late systolic regurgitation due to prolapse of the mitral leaflet(s) into the left atrium. Such late systolic murmurs can also occur in the absence of clicks.

Early diastolic murmurs begin with or shortly after S2, when the associated ventricular pressure drops sufficiently below that in the aorta or pulmonary artery. High-pitched murmurs of aortic regurgitation (AR) or pulmonic regurgitation due to pulmonary hypertension are generally decrescendo, consistent with the rapid decline in volume or rate of regurgitation during diastole. The diastolic murmur of pulmonic regurgitation without pulmonary hypertension is low to medium pitched, and the onset of this murmur is slightly delayed because regurgitant flow is minimal at pulmonic valve closure, when the reverse pressure gradient responsible for the regurgitation is minimal. Such murmurs are common late after repair of tetralogy of Fallot.

Middiastolic murmurs usually originate from the mitral and tricuspid valves, occur early during ventricular filling, and are due to a relative disproportion between valve orifice size and diastolic blood flow volume. Although they are usually due to mitral or tricuspid stenosis, middiastolic murmurs may also be due to increased diastolic blood flow across the mitral or tricuspid valve when such valves are severely regurgitant, across the normal mitral valve (MV) in patients with ventricular septal defect or patent ductus arteriosus, and across the normal tricuspid valve in patients with atrial septal defect. In severe, chronic AR, a low-pitched, rumbling diastolic murmur (Austin-Flint murmur) is often present at the LV apex; it may be either middiastolic or presystolic. An opening snap is absent in isolated AR.

Presystolic murmurs begin during the period of ventricular filling that follows atrial contraction and therefore occur in sinus rhythm. They are usually due to mitral or tricuspid stenosis. A right or left atrial myxoma may cause either middiastolic or presystolic murmurs similar to tricuspid or mitral stenosis (MS).

Continuous murmurs arise from high- to low-pressure shunts that persist through the end of systole and the beginning of diastole. Thus, they begin in systole, peak near S2, and continue into all or part of diastole. There are many causes of continuous murmurs, but they are uncommon in patients with valvular heart disease.5–9

2.1.2.1. Dynamic Cardiac Auscultation
Attentive cardiac auscultation during dynamic changes in cardiac hemodynamics often enables the observer to deduce the correct origin and significance of a cardiac murmur.10–13 Changes in the intensity of heart murmurs during various maneuvers are indicated in Table 2.


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  		Table 2. Interventions Used to Alter the Intensity of Cardiac Murmurs

2.1.2.2. Other Physical Findings
The presence of other physical findings, either cardiac or noncardiac, may provide important clues to the significance of a cardiac murmur and the need for further testing (Fig. 2). for example, a right heart murmur in early to midsystole at the lower left sternal border likely represents TR without pulmonary hypertension in an injection drug user who presents with fever, petechiae, Osler’s nodes, and Janeway lesions.


Figure 213968
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  		Figure 2. Strategy for evaluating heart murmurs.

*If an electrocardiogram or chest X-ray has been obtained and is abnormal, echocardiography is indicated.

Associated cardiac findings frequently provide important information about cardiac murmurs. Fixed splitting of the second heart sound during inspiration and expiration in a patient with a grade 2/6 midsystolic murmur in the pulmonic area and left sternal border should suggest the possibility of an atrial septal defect. A soft or absent A2 or reversed splitting of S2 may denote severe AS. An early aortic systolic ejection sound heard during inspiration and expiration suggests a bicuspid aortic valve, whereas an ejection sound heard only in the pulmonic area and at the left sternal border during expiration usually denotes pulmonic valve stenosis. LV dilatation on precordial palpation and bibasilar pulmonary rales favor the diagnosis of severe, chronic MR in a patient with a grade 2/6 holosystolic murmur at the cardiac apex. A slow-rising, diminished arterial pulse suggests severe AS in a patient with a grade 2/6 midsystolic murmur at the second right intercostal space. The typical parvus et tardus pulse may be absent in the elderly, even in those with severe AS, secondary to the effects of aging on the vasculature. Pulsus parvus may also occur with severely reduced cardiac output from any cause. Factors that aid in the differential diagnosis of LV outflow tract obstruction are listed in Table 3.14 Examination of the jugular venous wave forms may provide additional or corroborative information. For example, regurgitant cv waves are indicative of TR and are often present without an audible murmur.


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  		Table 3. Factors That Differentiate the Various Causes of Left Ventricular Outflow Tract Obstruction

2.1.2.3. Associated Symptoms
An important consideration in the patient with a cardiac murmur is the presence or absence of symptoms15 (Fig. 2). For example, symptoms of syncope, angina pectoris, or heart failure in a patient with a midsystolic murmur will usually result in a more aggressive diagnostic approach than in a patient with a similar midsystolic murmur who has none of these symptoms. An echocardiogram to rule in or rule out the presence of significant AS should be obtained. A history of thromboembolism will also usually result in a more extensive workup. In patients with cardiac murmurs and clinical findings suggestive of endocarditis, echocardiography is indicated.2

Conversely, many asymptomatic children and young adults with grade 2/6 midsystolic murmurs and no other cardiac physical findings need no further workup after the initial history and physical examination (Fig. 2). A particularly important group is the large number of asymptomatic older patients, many with systemic hypertension, who have midsystolic murmurs, usually of grade 1 or 2 intensity, related to sclerotic aortic valve leaflets; flow into tortuous, noncompliant great vessels; or a combination of these findings. Such murmurs must be distinguished from those caused by more significant degrees of aortic valve thickening, calcification, and reduced excursion that result in milder or greater degrees of valvular AS. The absence of LV hypertrophy on the electrocardiogram (ECG) may be reassuring, but echocardiography is frequently necessary. Aortic sclerosis can be defined by focal areas of increased echogenicity and thickening of the leaflets without restriction of motion and a peak velocity of less than 2.0 m per second. The recognition of aortic valve sclerosis may prompt the initiation of more aggressive programs of coronary heart disease prevention. In patients with AS, it is difficult to assess the rate and severity of disease progression on the basis of auscultatory findings alone.

2.1.3. Electrocardiography and Chest Roentgenography
Although echocardiography usually provides more specific and often quantitative information about the significance of a heart murmur and may be the only test needed, the ECG and chest X-ray are readily available and may have been obtained previously. The absence of ventricular hypertrophy, atrial enlargement, arrhythmias, conduction abnormalities, prior myocardial infarction, and evidence of active ischemia on the ECG provides useful negative information at a relatively low cost. Abnormal ECG findings in a patient with a heart murmur, such as ventricular hypertrophy or a prior infarction, should lead to a more extensive evaluation that includes echocardiography (Fig. 2).

Posteroanterior and lateral chest roentgenograms often yield qualitative information on cardiac chamber size, pulmonary blood flow, pulmonary and systemic venous pressure, and cardiac calcification in patients with cardiac murmurs. When abnormal findings are present on chest X-ray, echocardiography should be performed (Fig. 2). A normal chest X-ray and ECG are likely in asymptomatic patients with isolated midsystolic murmurs, particularly in younger age groups, when the murmur is grade 2 or less in intensity and heard along the left sternal border.16–18 Routine ECG and chest radiography are not recommended in this setting.

2.1.4. Echocardiography
Class I

  1. Echocardiography is recommended for asymptomatic patients with diastolic murmurs, continuous murmurs, holosystolic murmurs, late systolic murmurs, murmurs associated with ejection clicks, or murmurs that radiate to the neck or back. (Level of Evidence: C)
  2. Echocardiography is recommended for patients with heart murmurs and symptoms or signs of heart failure, myocardial ischemia/infarction, syncope, thromboembolism, infective endocarditis, or other clinical evidence of structural heart disease. (Level of Evidence: C)
  3. Echocardiography is recommended for asymptomatic patients who have grade 3 or louder midpeaking systolic murmurs. (Level of Evidence: C)

Class IIa

  1. Echocardiography can be useful for the evaluation of asymptomatic patients with murmurs associated with other abnormal cardiac physical findings or murmurs associated with an abnormal ECG or chest X-ray. (Level of Evidence: C)
  2. Echocardiography can be useful for patients whose symptoms and/or signs are likely noncardiac in origin but in whom a cardiac basis cannot be excluded by standard evaluation. (Level of Evidence: C)

Class III
1. Echocardiography is not recommended for patients who have a grade 2 or softer midsystolic murmur identified as innocent or functional by an experienced observer. (Level of Evidence: C)

Echocardiography with color flow and spectral Doppler evaluation is an important noninvasive method for assessing the significance of cardiac murmurs. Information regarding valve morphology and function, chamber size, wall thickness, ventricular function, pulmonary and hepatic vein flow, and estimates of pulmonary artery pressures can be readily integrated.

Although echocardiography can provide important information, such testing is not necessary for all patients with cardiac murmurs and usually adds little but expense in the evaluation of asymptomatic younger patients with short grade 1 to 2 midsystolic murmurs and otherwise normal physical findings. At the other end of the spectrum are patients with heart murmurs for whom transthoracic echocardiography proves inadequate. Depending on the specific clinical circumstances, transesophageal echocardiography, cardiac magnetic resonance, or cardiac catheterization may be indicated for better characterization of the valvular lesion.

It is important to note that Doppler ultrasound devices are very sensitive and may detect trace or mild valvular regurgitation through structurally normal tricuspid and pulmonic valves in a large percentage of young, healthy subjects and through normal left-sided valves (particularly the MV) in a variable but lower percentage of patients.16,19–22

General recommendations for performing echocardiography in patients with heart murmurs are provided. Of course, individual exceptions to these indications may exist.

2.1.5. Cardiac Catheterization
Cardiac catheterization can provide important information about the presence and severity of valvular obstruction, valvular regurgitation, and intracardiac shunting. It is not necessary in most patients with cardiac murmurs and normal or diagnostic echocardiograms, but it provides additional information for some patients in whom there is a discrepancy between the echocardiographic and clinical findings. Indications for cardiac catheterization for hemodynamic assessment of specific valve lesions are given in Section 3, "Specific Valve Lesions," in these guidelines. Specific indications for coronary angiography to screen for the presence of CAD are given in Section 10.2.

2.1.6. Exercise Testing
Exercise testing can provide valuable information in patients with valvular heart disease, especially in those whose symptoms are difficult to assess. It can be combined with echocardiography, radionuclide angiography, and cardiac catheterization. It has a proven track record of safety, even among asymptomatic patients with severe AS. Exercise testing has generally been underutilized in this patient population and should constitute an important component of the evaluation process.

2.1.7. Approach to the Patient
The evaluation of the patient with a heart murmur may vary greatly depending on many of the considerations discussed above.23,24 These include the timing of the murmur in the cardiac cycle, its location and radiation, and its response to various physiological maneuvers (Table 2). Also of importance is the presence or absence of cardiac and noncardiac symptoms and other findings on physical examination that suggest the murmur is clinically significant (Fig. 2).

Patients with diastolic or continuous heart murmurs not due to a cervical venous hum or a mammary souffle during pregnancy are candidates for echocardiography. If the results of echocardiography indicate significant heart disease, further evaluation may be indicated. An echocardiographic examination is also recommended for patients with apical or left sternal edge holosystolic or late systolic murmurs, for patients with midsystolic murmurs of grade 3 or greater intensity, and for patients with softer systolic murmurs in whom dynamic cardiac auscultation suggests a definite diagnosis (e.g., hypertrophic cardiomyopathy).

Echocardiography is also recommended for patients in whom the intensity of a systolic murmur increases during the Valsalva maneuver, becomes louder when the patient assumes the upright position, and decreases in intensity when the patient squats. These responses suggest the diagnosis of either hypertrophic obstructive cardiomyopathy or MV prolapse (MVP). Additionally, further assessment is indicated when a systolic murmur increases in intensity during transient arterial occlusion, becomes louder during sustained handgrip exercise, or does not increase in intensity either in the cardiac cycle that follows a premature ventricular contraction or after a long R-R interval in patients with atrial fibrillation. The diagnosis of MR or ventricular septal defect in these circumstances is likely.

In many patients with grade 1 or 2 midsystolic murmurs, an extensive workup is not necessary. This is particularly true for children and young adults who are asymptomatic, have an otherwise normal cardiac examination, and have no other physical findings associated with cardiac disease.

However, echocardiography is indicated in certain patients with grade 1 or 2 midsystolic murmurs, including patients with symptoms or signs consistent with infective endocarditis, thromboembolism, heart failure, myocardial ischemia/infarction, or syncope. Echocardiography also usually provides an accurate diagnosis in patients with other abnormal physical findings, including widely split second heart sounds, systolic ejection sounds, and specific changes in