ACC/AHA Guidelines for Valve Disease

Summary / Explanation

The 2022 American College of Cardiology/American Heart Association (ACC/AHA) guidelines on valvular heart disease were written by a joint committee considering recommendations from all previous guidelines. Below is a summary of the key recommendations from the guidelines.[1]

Introduction

The initial evaluation for all patients with valvular heart disease is based on history and physical examination, electrocardiogram, transthoracic echocardiogram (TTE), and chest radiograph. Biomarkers, frailty scores, transesophageal echocardiogram (TEE), stress testing, cardiac magnetic resonance imaging, and cardiac catheterization can also improve diagnostic evaluation and risk stratification of VHD.

Valvular heart disease is classified based on the following stages:

• Stage A – At risk
• Stage B – Progressive
• Stage C – Asymptomatic severe
• Stage D – Symptomatic severe

Stage D valvular heart disease surgical management risk assessment should be performed using the Society of Thoracic Surgeons (STS) score, frailty indices, or the EUROSCORE II risk calculator.

Medical Therapy 

Primary antibiotic prophylaxis is recommended in patients with rheumatic fever to prevent valvular heart disease for over or equal to 10 years or until age 40 (whichever is longer). Antibiotic prophylaxis for infective endocarditis is recommended before dental procedures for patients with prosthetic valves and prosthetic valve repair (rings, chords, or clips), prior infective endocarditis, unrepaired congenital heart disease or repaired congenital heart disease with residual shunts, and cardiac transplant with valvular regurgitation.

The duration of antibiotic therapy for secondary prophylaxis of rheumatic fever depends on the presence or absence of valvular heart disease, carditis, and the risk of Group A streptococcus exposure.

Oral anticoagulation with non-vitamin K antagonists is recommended for patients with atrial fibrillation and valvular heart disease based on CHA₂DS₂VASc score, except for patients with mechanical prosthetic valves, rheumatic mitral stenosis and bioprosthetic prosthetic valve implantation less than or equal to 3 months ago, where vitamin K antagonist oral anticoagulation is recommended. In patients with asymptomatic aortic stenosis (AS), hypertension should be treated. Statin therapy is indicated for primary and secondary prevention of atherosclerosis in all patients with calcific AS.

Aortic Valve Disease

• Stage D AS is classified as:
  • High gradient AS
  • Low flow, low gradient AS
  • Paradoxical low flow low gradient AS

• In patients with suspected low-flow, low-gradient AS, low-dose dobutamine stress echocardiography, aortic valve calcium score using computed tomography (CT), and dimensionless index can be used to assess the severity. Before severity assessment, blood pressure control is required in patients with suspected paradoxical low-flow, low-gradient AS.

• In asymptomatic severe AS, exercise testing can be used to look for physiological changes and symptoms. Intervention is recommended with abnormal findings.

• Aortic valve replacement (AVR) is recommended in stage D AS, stage C AS with left ventricular ejection fraction (LVEF) less than 50%, and stage B AS undergoing other cardiac surgery. AVR is also reasonable for patients with very severe AS, decreased exercise tolerance or hypotension during exercise testing, serum B-type natriuretic peptide greater than 3 times normal, and an increase in aortic velocity greater than or equal to 0.3 m/s per year.

• In patients requiring AVR, surgical versus transcatheter AVR is decided based on patient age, life expectancy, open surgical risk, and anatomical considerations. Surgical AVR (SAVR) is recommended for patients aged under 65 with a life expectancy greater than 20 years. For patients aged 65 to 80, SAVR vs. transcatheter aortic valve intervention (TAVI) is recommended based on anatomy, risk, and shared decision-making. For patients aged over 80, with a life expectancy of less than 10 years or high/prohibitive surgical risk, TAVI is recommended if expected post-intervention survival is greater than 12 months with acceptable quality of life.

• In patients undergoing surgical AVR, the choice for a mechanical or bioprosthetic prosthetic valve is based on their age, the potential need for and risks associated with valve reintervention, the ability to take vitamin K antagonist oral anticoagulant, and patient preferences. In patients aged less than 50 with the appropriate anatomy, aortic valve replacement with pulmonic autograft (Ross procedure) may be considered at a comprehensive valve center.

• In chronic severe aortic regurgitation (AR), AVR is indicated in patients with stage D AR and stage C AR with LVEF less than or equal to 50%, left ventricular end-systolic volume (LVESV) greater than 50 mm or indexed left ventricular end-systolic volume (LVESVi) greater than 25 mm/m².

• In patients with bicuspid aortic valve, echocardiography should be used to evaluate AS and AR, and CT angiography and/or MR angiography should be used to assess aortic pathology. Surgical management of a bicuspid aortic valve should be performed for asymptomatic or symptomatic patients in conjunction with aortic replacement when the aortic sinus or ascending aortic diameter is greater than 5.5 cm.

Mitral Valve Disease

• Percutaneous mitral balloon commissurotomy (PMBC) is recommended in patients with stage D (valve area less than 1.5 cm²) rheumatic mitral stenosis (MS) who have favorable anatomy, less than moderate mitral regurgitation (MR) and absence of left atrial thrombus. PMBC can also be considered in patients with stage C MS with pulmonary artery systolic pressure (PASP) greater than 50 mm Hg. Surgical mitral valve intervention is recommended in severe rheumatic MS in those who are not candidates for PMBC, have failed PMBC, require other cardiac procedures, or do not have access to PMBC.

• Exercise testing is recommended for patients with rheumatic MS after a discrepancy between symptoms and echocardiographic findings to observe changes in exercise tolerance, PASP, and transmitral gradient.

• In patients with primary or secondary MR, in addition to TTE, TEE can be used to evaluate MR severity and the mechanism of MR. In contrast, cardiac magnetic resonance imaging can accurately evaluate left and right ventricular function and volumes. For asymptomatic patients with severe primary MR, TTE is indicated every 6 to 12 months to monitor LV function and pulmonary artery pressure. In secondary MR, cardiac catheterization, coronary CT angiography, and cardiac magnetic resonance imaging can be used to assess ventricular function and viability. TEE is used mainly for preprocedural planning for transcatheter-edge-to-edge repair (TEER) and intraprocedural imaging.

• Mitral valve intervention is indicated in patients with stage D MR or asymptomatic/stage C MR with LVEF less than or equal to 60% or LVESD greater than 40 mm. In patients requiring mitral valve surgery for primary MR, mitral valve repair is preferred over mitral valve replacement if the anatomic cause is a degenerative disease and a successful, durable repair is possible. In primary MR requiring mitral valve intervention who have high surgical risk, TEER can be considered.

• In patients with stage D secondary primary MR, as well as select patients with secondary MR remaining severely symptomatic despite optimal medical therapy for heart failure, TEER can be considered for LVEF 20% to 50%, LVESV less than or equal to 70 mm, and PASP less than 70 mm Hg and are at high or prohibitive risk for surgery. TEE is indicated to determine the procedure's suitability. Mitral valve surgery can be considered in stage D secondary MR when LVEF is greater than or equal to 50% for those with severe persistent symptoms as well as those with chronic severe secondary MR related to LV systolic dysfunction (LVEF less than 50%) while on optimal guideline-directed medical therapy for heart failure.

Tricuspid Valve Disease

Secondary tricuspid regurgitation (TR) should be managed medically to treat the etiology (eg, heart failure, atrial fibrillation, pulmonary hypertension). Tricuspid valve surgery is indicated for patients with stage C and D TR who are undergoing left-sided valve surgery and may be beneficial for stage B TR with annular dilation (tricuspid end-diastolic diameter greater than 40 mm or greater than 21 mm/m² indexed to body surface area). Isolated tricuspid valve surgery is also reasonable in patients who have stage D primary TR with evidence of right-sided heart failure refractory to medical therapy if completed before the onset of severe RV dysfunction or end-organ liver and kidney damage.

Prosthetic Valves

When suspicion of prosthetic valve dysfunction with a TTE does not show evidence of the same, 3-dimensional TEE, gated cardiac CT, or fluoroscopy is indicated to assess prosthetic valve dysfunction, as TTE may be limited by acoustic shadowing from the prosthesis. Surveillance TTE imaging for a surgical bioprosthesis is once every 5 to 10 years and then annually after implantation, even without a clinical change. Annual TTE for a transcatheter aortic bioprosthesis is reasonable.

The decision to use a mechanical versus a bioprosthetic surgical valve should involve shared decision-making and consider the patient's age, life expectancy, risk of long-term oral anticoagulation, risk of reintervention, anatomical considerations, access to medical care, and patient preference. For patients with mechanical valves undergoing minor procedures (eg, dental extraction, cataract surgery), vitamin K antagonist anticoagulation should be continued. For patients with bileaflet mechanical valves and no other thromboembolism risk factor undergoing invasive procedures, temporary interruption of the anticoagulation without bridging therapy is recommended. For patients with bioprosthetic heart valves and atrial fibrillation with a high CHA₂DS₂VASc score, patients with a mechanical mitral valve, and patients with a mechanical aortic valve with significant risk for thromboembolism, bridging therapy should be considered, when the INR is subtherapeutic. Increasing the INR goal or adding low-dose aspirin is reasonable in patients with a mechanical heart valve and a systemic embolic event on therapeutic INR.

For patients who have thrombosis of left-sided mechanical valves, urgent management with fibrinolytic therapy or emergent surgery is recommended. Anticoagulation with vitamin K is recommended for patients with thrombosis of bioprosthetic valves.

For patients with symptomatic prosthetic valve stenosis, repeat surgical or transcatheter intervention is indicated depending on surgical risk. For patients with paravalvular regurgitation or severe valvular regurgitation causing intractable hemolysis or NYHA Class III or IV heart failure symptoms, surgical reintervention is recommended. For those with a high surgical risk and favorable anatomy, transcatheter paravalvular leak repair or a valve-in-valve procedure is reasonable.

Infective Endocarditis

The Modified Duke criteria should be used to diagnose suspected infective endocarditis (IE). TTE is the first-line imaging test for IE, and TEE is indicated for patients with nondiagnostic TTE with prosthetic valves or intracardiac device leads when suspicion of complications from IE or for staphylococcal, enterococcal, or fungal bacteremia are apparent. CT imaging is reasonable in patients without clearly delineated anatomy with echocardiography in the setting of suspected paravalvular infection. ¹⁸F-fluorodeoxyglucose PET/CT can be used as an adjunct for diagnosing IE. Intravenous antibiotics are first-line therapy for all patients with IE.

Early surgical intervention (during initial hospitalization and before completion of the therapeutic course of antibiotics) is recommended in patients with valve dysfunction causing heart failure, complicated IE (ie, heart block, annular or periaortic abscess, destructive penetrating lesions), patients with persistent bacteremia (greater than 5 days after initiation of appropriate antimicrobial therapy), in patients with IE due to S aureus, fungal infection, or highly resistant organisms. Surgical intervention is also reasonable in patients with relapsing prosthetic valve endocarditis or recurrent emboli and persistent vegetation despite antibiotic therapy. The pacemaker or defibrillator should be removed for patients with IE and implanted cardiac electronic devices, including all the leads and the generator.

Patients with native left-sided valve mobile vegetations greater than 10 mm in early surgery may be considered. In patients with an indication for surgery who have suffered a stroke but have no evidence of intracranial hemorrhage or extensive neurological damage, operation without delay is recommended. For those with extensive neurologic damage, major ischemic stroke, or bleeding, delaying surgery at least 4 weeks may be considered in hemodynamically stable patients.

Pregnancy and Valvular Heart Disease 

All pregnant women with stage C and D valvular heart disease should undergo pre-pregnancy counseling and should be monitored at a tertiary care center with a heart valve team. Exercise testing is reasonable for stage C valvular heart disease pre-conception. Women with stage D valvular heart disease should undergo valve intervention before pregnancy. The choice of prosthetic valves should be based on a shared decision-making process that accounts for the patient's values and preferences, including discussing the risks of a mechanical valve during pregnancy and the reduced durability of bioprosthetic valves in young women. Women with stage C MS should undergo PMBC before pregnancy. Women with stage C high gradient AS should undergo valve intervention before pregnancy.

In pregnant women with stage D AS, valve intervention is reasonable when hemodynamic deterioration or heart failure symptoms are present. In pregnant women with stage D MS, PMBC is reasonable when symptoms persist after medical therapy. In pregnant women with stage D MR refractory to medical therapy, valve intervention is reasonable during pregnancy. For pregnant women with mechanical valves who require a warfarin dose of less than or equal to 5 mg/day, warfarin can be continued for all 3 trimesters after a discussion of risks and benefits with the patient.

For pregnant women with mechanical valves who require a warfarin dose of greater than 5 mg/day, dose-adjusted low molecular weight heparin is recommended during the first trimester, followed by the resumption of warfarin during the second and third trimesters. Pregnant women with mechanical heart valves who are on warfarin should be switched to twice-daily low molecular weight heparin at least 1 week before planned delivery and switched to intravenous unfractionated heparin at least 36 hours before scheduled delivery, which should be stopped at least 6 hours before delivery. For pregnant women with thrombosis of a mechanical valve, slow infusion low-dose fibrinolytic therapy is reasonable.

Concomitant Cardiac Conditions

For patients undergoing transcatheter aortic valve intervention, a coronary evaluation using invasive angiography or coronary CT angiography (for patients with low pretest probability) is recommended to guide potential revascularization of left main or proximal coronary artery disease (CAD). In patients planned for valve intervention, with anginal symptoms, evidence of ischemia, left ventricular systolic dysfunction, history of CAD, or risk factors for CAD, coronary evaluation is recommended before valve intervention, and revascularization is reasonable for those with left main or proximal CAD. In patients with atrial fibrillation and severe valvular heart disease planned for valvular surgery, surgical pulmonary vein isolation or a maze procedure, as well as surgical left atrial appendage ligation or excision, should be considered.

The guidelines promote a multidisciplinary heart team evaluation for patients with severe valvular heart disease comprising clinical cardiologists with expertise, advanced cardiac imaging specialists, interventional cardiologists, advanced heart failure specialists, and cardiac valve surgeons.