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JOURNAL：ACC
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2020 ACC/AHA Heart Valve Disease Guideline: Key Perspectives
D Mukherjee, NM Bhave,S Kohnstamm.

KEYWORDS
valvular heart disease ; guideline; TAVR

The 2020 guideline for the management of patients with valvular heart disease replaces the American Heart Association/American College of Cardiology (AHA/ACC) 2014 guideline and the 2017 focused update.


The following is part I of 3 key perspectives regarding some general considerations; and regarding patients with aortic stenosis (AS), aortic regurgitation (AR), or bicuspid aortic valve (BAV):

  1. 1. The guideline continues to recommend the use of disease stages among patients with valvular heart disease, consisting of Stage A (at risk), Stage B (progressive), Stage C (asymptomatic severe; with ventricular compensation [Stage C1] or with ventricular decompensation [Stage C2]), and Stage D (symptomatic severe). Disease stages should be assigned based on valve anatomy, the severity of valve dysfunction, the ventricular and pulmonary circulation response to valve dysfunction, and symptoms.
  2. 2. Among patients with atrial fibrillation and native heart valve disease other than rheumatic mitral stenosis, or among patients with atrial fibrillation and a bioprosthesis >3 months after valve replacement, a non–vitamin K oral anticoagulant (NOAC) is an effective alternative to anticoagulation with a vitamin K antagonist (VKA); among these patients, either a NOAC or VKA should be used based on the CHA2DS2-VASc score. Anticoagulation with a VKA should be used among patients with atrial fibrillation and rheumatic mitral stenosis. A NOAC should not be used in patients with a mechanical prosthesis without or with atrial fibrillation.
  3. 3. All patients with severe valvular heart disease being considered for intervention should be evaluated by a Multidisciplinary Heart Valve Team. Consultation with or referral to a Primary Valve Center or a Comprehensive Valve Center is reasonable for the discussion of treatment options in the setting of asymptomatic patients with severe valve disease, patients who might benefit from valve repair rather than valve replacement, and among patients with multiple comorbidities.
  4. 4. Among patients with severe symptomatic (Stage D) AS, the disease is subcategorized based on the gradient, flow, and left ventricular ejection fraction (LVEF). Stage D1 reflects patients with high-gradient symptomatic AS (Vmax ≥4.0 m/s, mean gradient ≥40 mm Hg, aortic valve area [AVA] ≤1.0 cm2); Stage D2 reflects low-flow, low-gradient severe AS with reduced LVEF (AVA ≤1.0 cm2, Vmax <4.0 m/s or mean gradient <40 mm Hg, LVEF <50%); and Stage D3 reflects low-flow, low-gradient severe AS with normal LVEF (“paradoxical low-flow severe AS”; LVEF ≥50%, stroke volume index <35 ml/m2).
  5. 5. Intervention for severe AS predominantly is based on the presence of symptoms or LV systolic dysfunction (Class 1); or in asymptomatic patients at low surgical risk with decreasing exercise tolerance or exercise-associated decrease ≥10 mm Hg in systolic blood pressure, very severe AS (Vmax ≥5.0 m/s), serum B-type natriuretic peptide (BNP) >3 times normal, or progression of Vmax ≥0.3 m/s per year (Class 2a). In addition, intervention can be considered among asymptomatic patients with severe high-gradient AS and a progressive decrease in LVEF to <60% on ≥3 serial imaging studies (Class 2b).
  6. 6. Among patients in whom a bioprosthesis is appropriate, decisions between surgical aortic valve replacement (SAVR) and transcatheter aortic valve implantation (TAVI) should include the presence of symptoms, patient age and anticipated life expectancy, the indication for intervention, predicted surgical risk, and anatomy or other factors referable to transfemoral (TF) TAVI feasibility (all Class 1):
    • SAVR is preferred among patients <65 years="" of="" age="" or="" with="" life="" expectancy="">20 years.
    • SAVR is preferred if vascular anatomy or other factors preclude TF TAVI.
    • SAVR is preferred among asymptomatic patients with a Class 2a indication for intervention, such as an abnormal exercise test, very severe AS, rapid progression, or elevated BNP.
    • If feasible, TF TAVI is preferred among patients >80 years of age or with life expectancy <10 years.
    • SAVR or TF TAVI is recommended after shared decision making among symptomatic patients ages 65-80 years with no contraindication to TF TAVI.
    • TAVI is preferred among symptomatic patients of any age with high or prohibitive surgical risk, if predicted survival after intervention is >12 months with an acceptable quality of life.
    • After shared decision making, palliative care is recommended among symptomatic patients with predicted post-TAVI survival <12 months or for whom minimal improvement in quality of life is expected.
  7. 7. Among patients with asymptomatic severe (Stage C) AR, the disease is subcategorized based on LVEF and LV end-systolic diameter (LVESD). Stage C1 reflects normal LVEF (≥55%; previously ≥50% in the 2014 AHA/ACC guidelines) and mild to moderate LV dilation (LVESD <50 mm). Stage C2 reflects abnormal LV systolic function (LVEF <55%; previously <50%) or severe LV dilation (LVESD 50 mm or indexed LVESD >25 mm/m2; unchanged from previous).
  8. 8. Intervention for severe AR is based on the presence of symptoms or LV systolic dysfunction (LVEF ≤55%; both Class 1); or the presence of severe LV dilation (LVESD >50 mm or indexed LVESD >25 mm/m2; Class 2a).
  9. 9. Among patients with BAV, transthoracic echocardiography is recommended to assess valve morphology, assess AS and AR, assess the aortic root and ascending aorta, and evaluate for the presence of aortic coarctation. If the aortic sinuses, sinotubular junction, and ascending aorta cannot be accurately or fully assessed on echocardiography, then cardiac magnetic resonance angiography or computed tomography angiography is indicated. Lifelong serial imaging is indicated if the aorta diameter is ≥4.0 cm.
  10. 10. Among patients with BAV, indications for replacement of the aorta remain similar to previous: aortic diameter >5.5 cm (Class 1), aortic diameter 5.0-5.5 cm plus an additional risk factor for dissection (family history of dissection, aortic growth >0.5 cm per year, aortic coarctation; Class 2a), or aortic diameter ≥4.5 cm with an indication for SAVR (Class 2a).

The following is Part 2 of 3 key perspectives regarding mitral stenosis (MS), mitral regurgitation (MR), and tricuspid valve disease:


  1. 1. Recurrent rheumatic fever is associated with worsening of rheumatic heart disease. Therefore, for secondary prevention of rheumatic fever, in patients with previous episodes of rheumatic fever or evidence of rheumatic heart disease, long-term antistreptococcal prophylaxis is indicated (Class 1 recommendation). Antibiotic options include penicillin, sulfadiazine, and macrolides. The recommended durations of prophylaxis are as follows:
    • Rheumatic fever with carditis and residual valvular disease: 10 years or until patient is ≥40 years of age (whichever is longer)
    • Rheumatic fever with carditis but no residual valvular disease: 10 years or until patient is ≥21 years of age (whichever is longer)
    • Rheumatic fever without carditis: 5 years or until patient is ≥21 years of age (whichever is longer)
  2. 2. Rheumatic MS is much more common in women than in men (80% of cases in women). Worldwide, most MS is rheumatic, though calcific MS is becoming more common in the elderly population in high-income countries.
  3. 3. In patients with Stage D rheumatic MS (symptomatic MS with mitral valve area ≤1.5 cm2 and/or diastolic pressure half-time ≥150 ms, typically with mean mitral valve gradient >5-10 mm Hg) and favorable valve morphology with less than moderate MR and no left atrial appendage thrombus, percutaneous mitral balloon commissurotomy (PMBC) is recommended if it can be performed at a Comprehensive Valve Center (Class 1). If PMBC is not an option due to anatomic considerations, severe MR, or failed prior PMBC, surgical intervention is recommended, unless risk is prohibitive.
  4. 4. Patients with calcific MS often have multiple comorbidities and are of advanced age. Because stenosis results from mitral annular calcification encroaching on the leaflet bases, without involvement of the leaflet tips, PMBC is not beneficial. Severe mitral annular calcification can make secure implantation of a surgical prosthesis challenging and may result in residual MS following valve replacement. Therefore, in Stage D calcific MS, surgical intervention should be undertaken only after careful consideration of risks and potential benefits (Class 2b).
  5. 5. Timing of intervention for chronic primary MR should be based on symptoms and left ventricular (LV) size and function. Recommendations for surgical intervention for MR remain similar to those in the 2014 guideline. For severe primary MR due to degenerative mitral valve disease, surgical repair is recommended in preference to valve replacement, provided that a successful and durable repair is technically feasible (Class 1). In asymptomatic patients, surgery is recommended if LV ejection fraction (LVEF) ≤60% and/or LV end-systolic diameter ≥40 mm (Stage C2, Class 1 recommendation). Surgery may be considered if these LV criteria are not met but the likelihood of a successful and durable repair is >95% with <1% expected mortality at a Primary or Comprehensive Valve Center. Transcatheter edge-to-edge repair (TEER) outcomes in severe primary MR are inferior to those of surgical mitral valve repair, but TEER is a reasonable option if surgical risk is high or prohibitive and anatomy is favorable.
  6. 6. For secondary MR in the setting of LV dysfunction, guideline-directed medical therapy for heart failure is the mainstay of treatment, and secondary MR often improves with medical optimization.
  7. 7. In patients with severe secondary MR with LVEF 20-50%, LV end-systolic diameter ≤70 mm, and pulmonary artery systolic pressure <70 mm Hg who remain symptomatic after medical optimization for heart failure, TEER is reasonable if anatomy is favorable (Class 2a). Surgical intervention for severe secondary MR is reasonable if performed concomitantly with coronary artery bypass grafting (Class 2a). For secondary MR in the setting of atrial annular dilation in the setting of atrial fibrillation with preserved LV systolic function, if symptoms persist despite heart failure optimization and arrhythmia management, mitral valve surgery may be considered (Class 2b).
  8. 8. Tricuspid regurgitation (TR) is most often secondary, due to annular dilation in the setting of right ventricular (RV) dilation and/or dysfunction, as seen in pulmonary hypertension, or in right atrial dilation in the setting of atrial fibrillation. Medical treatment for TR consists of diuresis and treatment of underlying causes of heart failure and pulmonary hypertension.
  9. 9. In severe primary TR with symptoms of right heart failure, isolated tricuspid valve surgery can improve symptoms and reduce hospitalizations (Class 2a). In asymptomatic patients with severe primary TR, tricuspid valve surgery may be considered if RV dilation or dysfunction develop (Class 2b). With respect to secondary TR, patients undergoing left-sided valve surgery should be considered for concomitant tricuspid valve surgery if the TR is severe (Class 1), or if the tricuspid annulus is dilated (>4.0 cm) and/or if right heart failure symptoms have occurred (Class 2a). In symptomatic patients with severe, functional TR (particularly if related to atrial fibrillation and atrial dilation), surgical intervention is reasonable in the absence of severe RV dysfunction, pulmonary hypertension, and liver/kidney damage (Class 2a).
The following is Part 3 of 3 key perspectives regarding mixed valve disease, prosthetic valves, infective endocarditis (IE), and pregnancy and valvular heart disease:

Mixed Valve Disease

  1. 1. Management of mixed valve disease should follow the guidelines for the predominant lesion. For ambiguous symptoms, use of biomarkers or invasive hemodynamics at rest or exercise should be considered as adjuncts to traditional imaging. For patients with mixed moderate aortic stenosis/aortic regurgitation who have developed left ventricular (LV) dysfunction (LV ejection fraction [LVEF] <50%), surgical aortic valve replacement (SAVR) is indicated (Class 1).

Prosthetic Valves

  1. 2. Following an initial post-procedure transthoracic echocardiogram (TTE), surveillance imaging is recommended at 5 and 10 years, then annually. When there is a change in clinical symptoms or signs suggesting valve dysfunction, additional imaging such as transesophageal echocardiography (TEE), fluoroscopy, or cardiac computed tomography (CT) is recommended. For a bioprosthetic transcatheter aortic valve implantation (TAVI), annual TTE is reasonable.
  2. 3. Antithrombotic therapy for prosthetic valves:
    • Mechanical valves: Anticoagulation with vitamin K antagonists (VKAs) is recommended to achieve varying international normalized ratios (INRs) dependent on valve characteristics and patient risk factors (all Class 1).
    • For mechanical AVR with thromboembolic risk factors (hypercoagulable state, LV dysfunction, prior thromboembolism) or an older-generation prosthesis: INR of 3.0.
    • For mechanical bileaflet or current-generation single-tilting disk AVR with no risk factors: INR of 2.5.
    • For mechanical On-X AVR and no thromboembolic risk factors: A lower INR of 1.5-2.0, starting 3 months after surgery with addition of aspirin (ASA) 75-100 mg daily (Class 2b).
    • For mechanical mitral valve replacement: INR of 3.0.
    • Bioprosthetic TAVI/SAVR or mitral valve replacement: ASA 75-100 mg is reasonable (Class 2a).
    • Bioprosthetic SAVR or mitral valve replacement: Anticoagulation with VKA to an INR of 2.5 is reasonable for 3-6 months postoperatively (Class 2b).
    • Mechanical valve prosthesis: The use of direct thrombin inhibitors (dabigatran) or anti-Xa direct oral anticoagulants remains a Class 3 contraindication.
    • Bridging of a mechanical bileaflet aortic valve without other risk factors is not required, while those with mechanical AVR with thromboembolic risk factors, older-generation mechanical AVRs, or mechanical mitral valve replacements, all need bridging anticoagulation therapy (Class 2a).
  3. 4. For high surgical risk patients with prosthetic valve dysfunction (stenosis or valve regurgitation), a transcatheter valve-in-valve procedure is reasonable at a Comprehensive Valve Center (Class 2a). For high surgical risk patients with paravalvular regurgitation with intractable hemolysis or New York Heart Association (NYHA) class III or IV symptoms, a catheter-based percutaneous repair is reasonable (Class 2a).

Infective Endocarditis

  1. 5. Patients with IE should be managed by a multispecialty Heart Valve Team including an infectious disease specialist, as well as a neurologist for those who have had neurologic events. In patients with suspected IE, initial TTE is recommended to evaluate for the presence of a vegetation and its possible effects on valvular function (Class 1). Subsequent TEE is indicated when TTE is nondiagnostic, or when complications are suspected, or when intracardiac device leads are present (Class 1). TEE is reasonable in patients with S. aureus bacteremia without a known source, as well as patients with prosthetic valves and persistent fever without bacteremia (Class 2a). The newer imaging modality of 18F-fluorodeoxyglucose positron emission tomography /CT is now considered a valuable adjunct for patients classified by Modified Duke Criteria as having "possible IE" (Class 2a), particularly when prosthetic valves are not optimally visualized on standard TTE or TEE imaging.
  2. 6. Appropriate antibiotic therapy should be initiated after obtaining blood cultures and then tailored to antibiotic sensitivity data. Stable patients with left-sided IE caused by streptococcus, E. faecalis, S. aureus, or coagulase negative staph without evidence of paravalvular infect ion on TEE, can change to oral antibiotic therapy after initial intravenous (IV) therapy. A follow-up TEE is recommended 1-3 days prior to completion of the antibiotic course (Class 2b).
  3. 7. Early surgical intervention (during initial hospitalization and before completion of full therapeutic course of antibiotics) is recommended for patients with the following characteristics: heart failure symptoms; left-sided IE caused by S. aureus, fungal organism, or other highly resistant organisms; persistent bacteremia or fevers >5 days; recurrent emboli and persistent vegetation; native left-sided valve IE with a mobile vegetation >10 mm in length. In patients with prosthetic valve IE and relapsing infection after completion of an antibiotic course, surgery is recommended (Class 1).
  4. 8. In patients with IE with an indication for surgery who suffered a stroke without intracranial hemorrhage, surgery without delay may still be considered. However, if there has been extensive neurologic damage or intracranial hemorrhage, it is advised to wait ≥4 weeks (Class 2b).

Pregnancy and Valvular Heart Disease

  1. 9. Women with severe valve disease (Stages C and D) considering pregnancy should undergo pre pregnancy counseling and appropriate testing by a cardiologist with expertise in managing valvular heart disease in pregnancy with subsequent monitoring at a tertiary care center with a dedicated Heart Valve Team (Class 1).
  2. 10. In asymptomatic women considering pregnancy with either severe rheumatic mitral stenosis (MS) (mitral valve area ≤1.5 cm2, Stage C1) or severe aortic stenosis (aortic velocity ≥4.0 m/s or mean pressure gradient ≥40 mm Hg), valve intervention prior to conception is reasonable. For MS, a percutaneous mitral balloon commissurotomy should be considered if there is favorable valve morphology (Class 2a). If there is severe mitral regurgitation (Stage C1), and the valve is suitable to repair, this should be considered (Class 2b). For women undergoing valve replacement prior to pregnancy, the choice of the type of valve should be made through a shared decision-making process.
  3. 11. In pregnant women with either severe rheumatic MS, severe aortic stenosis, or severe mitral regurgitation with NHYA class III or IV symptoms refractory to medical therapy, intervention during pregnancy is reasonable (Class 2a).
  4. 12. Women with mechanical prosthetic valves have high-risk pregnancies and should be monitored closely. During the first trimester, warfarin is associated with the lowest likelihood of maternal complication but highest likelihood of miscarriage, fetal death, and congenial malformation, especially when the warfarin dose exceeds 5 mg/day.
  5. 13. Ideally, a switch to low molecular weight heparin (LMWH) (with target anti-Xa level of 0.8-1.2 U/ml 4-6 hours after dose) or IV unfractionated heparin (UFH) (with activated partial thromboplastin time [aPTT] 2x control) is made ≥1 week before planned delivery, followed by a switch to UFH ≥36 hours before planned delivery. UFH should be stopped ≥6 hours before planned vaginal delivery (Class 1). If urgent delivery via C-section is required, anticoagulation should be reversed first.
  6. 14. If warfarin <5 mg/day is required to maintain a therapeutic INR, continuation of warfarin for all three trimesters is reasonable (Class 2a), or dose-adjusted LMWH ≥2x/day during the first trimester, followed by warfarin for the second and third trimesters may be considered. If >5 mg/day is required, dose-adjusted LMWH during the first trimester, followed by warfarin for the second and third trimester, may be considered (Class 2a) or with continuation of LMWH for the second and third trimesters (Class 2b). The use of direct thrombin inhibitors and anti-Xa direct oral anticoagulants is still not recommended (Class 3).



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