2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary

A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines

1.1 Methodology and Evidence Review

The recommendations listed in this document are, whenever possible, evidence based. An extensive review was conducted on literature published through November 2012, and other selected references through October 2013 were reviewed by the guideline writing committee. The relevant data are included in evidence tables in the Data Supplement. Searches were extended to studies, reviews, and other evidence conducted on human subjects and that were published in English from PubMed, EMBASE, Cochrane, Agency for Healthcare Research and Quality Reports, and other selected databases relevant to this guideline. Key search words included but were not limited to the following: valvular heart disease, aortic stenosis, aortic regurgitation, bicuspid aortic valve, mitral stenosis, mitral regurgitation, tricuspid stenosis, tricuspid regurgitation, pulmonic stenosis, pulmonic regurgitation, prosthetic valves, anticoagulation therapy, infective endocarditis, cardiac surgery, and transcatheter aortic valve replacement. Additionally, the committee reviewed documents related to the subject matter previously published by the ACC and AHA. The references selected and published in this document are representative and not all-inclusive.

1.2 Organization of the Writing Committee

The committee was composed of clinicians, who included cardiologists, interventionalists, surgeons, and anesthesiologists. The committee included representatives from the American Association for Thoracic Surgery, American Society of Echocardiography (ASE), Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Anesthesiologists, and Society of Thoracic Surgeons (STS).

1.3 Document Review and Approval

This document was reviewed by 2 official reviewers each nominated by both the ACC and the AHA, as well as 1 reviewer each from the American Association for Thoracic Surgery, ASE, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Anesthesiologists, and STS and 39 individual content reviewers (which included representatives from the following ACC committees and councils: Adult Congenital and Pediatric Cardiology Section, Association of International Governors, Council on Clinical Practice, Cardiovascular Section Leadership Council, Geriatric Cardiology Section Leadership Council, Heart Failure and Transplant Council, Interventional Council, Lifelong Learning Oversight Committee, Prevention of Cardiovascular Disease Committee, and Surgeon Council). Reviewers' RWI information was distributed to the writing committee and is published in this document (Appendix 2).

This document was approved for publication by the governing bodies of the ACC and the AHA and endorsed by the American Association for Thoracic Surgery, ASE, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Anesthesiologists, and STS.

1.4 Scope of the Guideline

The focus of this guideline is the diagnosis and management of adult patients with valvular heart disease (VHD). A full revision of the original 1998 VHD guideline was made in 2006, and an update was made in 2008 (5). Some recommendations from the earlier VHD guidelines have been updated as warranted by new evidence or a better understanding of earlier evidence, whereas others that were inaccurate, irrelevant, or overlapping were deleted or modified. Throughout, our goal was to provide the clinician with concise, evidence-based, contemporary recommendations and the supporting documentation to encourage their use.

The full-text version of this guideline (4) was created in a different format from prior VHD guidelines to facilitate access to concise, relevant bytes of information at the point of care when clinical knowledge is needed the most. Thus, each COR is followed by a brief paragraph of supporting text and references. Where applicable, sections were divided into subsections of 1) diagnosis and follow-up, 2) medical therapy, and 3) intervention. The purpose of these subsections was to categorize the COR according to the clinical decision-making pathways that caregivers use in the management of patients with VHD. New recommendations for assessment of the severity of valve lesions have been proposed, based on current natural history studies of patients with VHD. The relevant data are included in evidence tables in the Data Supplement of the full-text guideline (4).

The present document applies to adult patients with VHD. Management of patients with congenital heart disease (CHD) and infants and children with valve disease are not addressed here. The document recommends a combination of lifestyle modifications and medications that constitute GDMT. Both for GDMT and other recommended drug treatment regimens, the reader is advised to confirm dosages with product insert material and to carefully evaluate for contraindications and drug–drug interactions. Table 2 is a list of associated guidelines that may be of interest to the reader. The table is intended for use as a resource and obviates the need to repeat extant guideline recommendations.

2.1 Evaluation of the Patient With Suspected VHD

Patients with VHD may present with a heart murmur, symptoms, or incidental findings of valvular abnormalities on chest imaging or noninvasive testing. Irrespective of the presentation, all patients with known or suspected VHD should undergo an initial meticulous history and physical examination, as well as a chest x-ray and electrocardiogram. A comprehensive transthoracic echocardiogram (TTE) with 2-dimensional imaging and Doppler interrogation should then be performed to correlate findings with initial impressions based on the initial clinical evaluation. The TTE will also be able to provide additional information, such as the effect of the valve lesion on the cardiac chambers and great vessels, and to assess for other concomitant valve lesions. Other ancillary testing such as transesophageal echocardiography (TEE), computed tomography (CT) or cardiac magnetic resonance (CMR) imaging, stress testing, and diagnostic hemodynamic cardiac catheterization may be required to determine the optimal treatment for a patient with VHD. An evaluation of the possible surgical risk for each individual patient should be performed if intervention is contemplated, as well as other contributing factors such as the presence and extent of comorbidities and frailty. Follow-up of these patients is important and should consist of an annual history and physical examination in most stable patients. An evaluation of the patient may be necessary sooner than annually if there is a change in the patient's symptoms. In some valve lesions there may be unpredictable adverse consequences on the left ventricle in the absence of symptoms necessitating more frequent follow-up. The frequency of repeat testing, such as echocardiography, will be dependent on the severity of the valve lesion and its effect on the left or right ventricle, coupled with the known natural history of the valve lesion.

2.2 Definitions of Severity of Valve Disease

Classification of the severity of valve lesions should be based on multiple criteria, including the initial findings on the physical examination, which should then be correlated with data from a comprehensive TTE. Intervention should primarily be performed on patients with severe VHD in addition to other criteria outlined in this document.

This document provides a classification of the progression of VHD with 4 stages (A to D) similar to that proposed by the “2013 ACCF/AHA Guideline for the Management of Heart Failure” (18). Indication for intervention in patients with VHD is dependent on 1) the presence or absence of symptoms; 2) the severity of VHD; 3) the response of the left and/or right ventricle to the volume or pressure overload caused by VHD; 4) the effect on the pulmonary or systemic circulation; and 5) a change in heart rhythm. The stages take into consideration all of these important factors (Table 3). The criteria for the stages of each individual valve lesion are listed in Section 3.1, Section 4.1, Section 6.1, Section 7.1, Section 8.1, Section 8.3, and Section 9.

The purpose of valvular intervention is to improve symptoms and/or prolong survival, as well as to minimize the risk of VHD-related complications such as asymptomatic irreversible ventricular dysfunction, pulmonary hypertension, stroke, and atrial fibrillation (AF). Thus, the criteria for “severe” VHD are based on studies describing the natural history of patients with unoperated VHD, as well as observational studies relating the onset of symptoms to measurements of severity. In patients with stenotic lesions, there is an additional category of “very severe” stenosis based on studies of the natural history showing that prognosis becomes poorer as the severity of stenosis increases.

2.3 Diagnostic Testing—Diagnosis and Follow-Up: Recommendations

See Table 4 for the frequency of echocardiograms in asymptomatic patients with VHD and normal left ventricular (LV) function.

• 1. TTE is recommended in the initial evaluation of patients with known or suspected VHD to confirm the diagnosis, establish etiology, determine severity, assess hemodynamic consequences, determine prognosis, and evaluate for timing of intervention (19–34). (Level of Evidence: B)

• 2. TTE is recommended in patients with known VHD with any change in symptoms or physical examination findings. (Level of Evidence: C)

• 3. Periodic monitoring with TTE is recommended in asymptomatic patients with known VHD at intervals depending on valve lesion, severity, ventricular size, and ventricular function. (Level of Evidence: C)

• 4. Cardiac catheterization for hemodynamic assessment is recommended in symptomatic patients when noninvasive tests are inconclusive or when there is a discrepancy between the findings on noninvasive testing and physical examination regarding severity of the valve lesion. (Level of Evidence: C)

• 1. Exercise testing is reasonable in selected patients with asymptomatic severe VHD to 1) confirm the absence of symptoms, or 2) assess the hemodynamic response to exercise, or 3) determine prognosis (35–39). (Level of Evidence: B)

2.4 Basic Principles of Medical Therapy: Recommendations

• 1. Secondary prevention of rheumatic fever is indicated in patients with rheumatic heart disease, specifically mitral stenosis (MS) (40). (Level of Evidence: C)

• 1. Prophylaxis against infective endocarditis (IE) is reasonable for the following patients at highest risk for adverse outcomes from IE before dental procedures that involve manipulation of gingival tissue, manipulation of the periapical region of teeth, or perforation of the oral mucosa (41–43) (Level of Evidence: B):

  • • Patients with prosthetic cardiac valves;

  • • Patients with previous IE;

  • • Cardiac transplant recipients with valve regurgitation due to a structurally abnormal valve; or

  • • Patients with CHD with:

    • ○ Unrepaired cyanotic CHD, including palliative shunts and conduits;

    • ○ Completely repaired congenital heart defect repaired with prosthetic material or device, whether placed by surgery or catheter intervention, during the first 6 months after the procedure; or

    • ○ Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device.

• 1. Prophylaxis against IE is not recommended in patients with VHD who are at risk of IE for nondental procedures (e.g., TEE, esophagogastroduodenoscopy, colonoscopy, or cystoscopy) in the absence of active infection (44). (Level of Evidence: B)

2.5 Evaluation of Surgical and Interventional Risk

See Table 5 for risk assessment combining STS risk estimate, frailty, major organ system dysfunction, and procedure-specific impediments.

2.6 The Heart Valve Team and Heart Valve Centers of Excellence: Recommendations

• 1. Patients with severe VHD should be evaluated by a multidisciplinary Heart Valve Team when intervention is considered. (Level of Evidence: C)

• 1. Consultation with or referral to a Heart Valve Center of Excellence is reasonable when discussing treatment options for 1) asymptomatic patients with severe VHD, 2) patients who may benefit from valve repair versus valve replacement, or 3) patients with multiple comorbidities for whom valve intervention is considered. (Level of Evidence: C)

A competent, practicing cardiologist should have the ability to diagnose and direct the treatment of most patients with VHD. For instance, otherwise healthy patients with severe VHD who become symptomatic should nearly always be considered for intervention. However, more complex decision-making processes may be required in select patient populations, such as those who have asymptomatic severe VHD, those who are at high risk for intervention, or those who could benefit from specialized therapies such as valve repair or transcatheter valve intervention.

The management of patients with complex severe VHD is best achieved by a Heart Valve Team composed primarily of a cardiologist and surgeon (including a structural valve interventionist if a catheter-based therapy is being considered). In selected cases, there may be a multidisciplinary, collaborative group of caregivers, including cardiologists, structural valve interventionalists, cardiovascular imaging specialists, cardiovascular surgeons, anesthesiologists, and nurses, all of whom have expertise in the management and outcomes of patients with complex VHD. The Heart Valve Team should optimize patient selection for available procedures through a comprehensive understanding of the risk–benefit ratio of different treatment strategies. This is particularly beneficial in patients in whom there are several options for treatment, such as the elderly high-risk patient with severe symptomatic aortic stenosis (AS) being considered for transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (AVR). The patient and family should be sufficiently educated by the Heart Valve Team about all alternatives for treatment so that their expectations can be met as fully as possible using a shared decision-making approach.

The optimal care of the patient with complex heart disease is best performed in centers that can provide all available options for diagnosis and management, including the expertise for complex aortic or mitral valve repair, aortic surgery, and transcatheter therapies. This has led to the development of Heart Valve Centers of Excellence. Heart Valve Centers of Excellence 1) are composed of experienced healthcare providers with expertise from multiple disciplines; 2) offer all available options for diagnosis and management, including complex valve repair, aortic surgery, and transcatheter therapies; 3) participate in regional or national outcome registries; 4) demonstrate adherence to national guidelines; 5) participate in continued evaluation and quality improvement processes to enhance patient outcomes; and 6) publicly report their available mortality and success rates. Decisions about intervention at the Heart Valve Centers of Excellence should be dependent on the centers' publicly available mortality rates and operative outcomes. It is recognized that some Heart Valve Centers of Excellence may have expertise in select valve problems.

3.1 Stages of Valvular AS

Medical and interventional approaches to the management of patients with valvular AS depend on accurate diagnosis of the cause and stage of the disease process. Table 6 shows the stages of AS ranging from patients at risk of AS (stage A) or with progressive hemodynamic obstruction (stage B) to severe asymptomatic (stage C) and symptomatic AS (stage D). Each of these stages is defined by valve anatomy, valve hemodynamics, the consequences of valve obstruction on the left ventricle and vasculature, as well as by patient symptoms. Hemodynamic severity is best characterized by the transaortic maximum velocity (or mean pressure gradient) when the transaortic volume flow rate is normal. However, some patients with AS have a low transaortic volume flow rate due to either LV systolic dysfunction with a low left ventricular ejection fraction (LVEF) or due to a small hypertrophied left ventricle with a low stroke volume. These categories of severe AS pose a diagnostic and management challenge distinctly different from the challenges faced by the majority of patients with AS, who have a high gradient and velocity when AS is severe. These special subgroups with low-flow AS are designated D2 (with a low LVEF) and D3 (with a normal LVEF).

The definition of severe AS is based on natural history studies of patients with unoperated AS, which show that the prognosis is poor once there is a peak aortic valve velocity of >4.0 m per second, corresponding to a mean aortic valve gradient >40 mm Hg. In patients with low forward flow, severe AS can be present with lower aortic valve velocities and lower aortic valve gradients. Thus, an aortic valve area should be calculated in these patients. The prognosis of patients with AS is poorer when the aortic valve area is <1.0 cm². At normal flow rates, an aortic valve area of <0.8 cm² correlates with a mean aortic valve gradient >40 mm Hg. However, symptomatic patients with a calcified aortic valve with reduced opening and an aortic valve area between 0.8 cm² and 1.0 cm² should be closely evaluated to determine whether they would benefit from valve intervention. Meticulous attention to detail is required when assessing aortic valve hemodynamics, either with Doppler echocardiography or cardiac catheterization, and the inherent variability of the measurements and calculations should always be considered in clinical-decision making.

3.2 Diagnosis and Follow-Up

The overall approach to the initial diagnosis of VHD is discussed in Section 2.3, and additional considerations specific to patients with AS are addressed here.

• 1. TTE is indicated in patients with signs or symptoms of AS or a bicuspid aortic valve for accurate diagnosis of the cause of AS, hemodynamic severity, LV size, and systolic function, and for determining prognosis and timing of valve intervention (26,27,45). (Level of Evidence: B)

• 1. Low-dose dobutamine stress testing using echocardiographic or invasive hemodynamic measurements is reasonable in patients with stage D2 AS with all of the following (46–48) (Level of Evidence: B):

  • a. Calcified aortic valve with reduced systolic opening;

  • b. LVEF less than 50%;

  • c. Calculated valve area 1.0 cm² or less; and

  • d. Aortic velocity less than 4.0 m per second or mean pressure gradient less than 40 mm Hg.

• 2. Exercise testing is reasonable to assess physiological changes with exercise and to confirm the absence of symptoms in asymptomatic patients with a calcified aortic valve and an aortic velocity 4.0 m per second or greater or mean pressure gradient 40 mm Hg or higher (stage C) (27,37,38,49). (Level of Evidence: B)

• 1. Exercise testing should not be performed in symptomatic patients with AS when the aortic velocity is 4.0 m per second or greater or mean pressure gradient is 40 mm Hg or higher (stage D) (50). (Level of Evidence: B)

3.3 Medical Therapy

• 1. Hypertension in patients at risk for developing AS (stage A) and in patients with asymptomatic AS (stages B and C) should be treated according to standard GDMT, started at a low dose, and gradually titrated upward as needed with frequent clinical monitoring (51–53). (Level of Evidence: B)

• 1. Vasodilator therapy may be reasonable if used with invasive hemodynamic monitoring in the acute management of patients with severe decompensated AS (stage D) with New York Heart Association (NYHA) class IV heart failure (HF) symptoms. (Level of Evidence: C)

• 1. Statin therapy is not indicated for prevention of hemodynamic progression of AS in patients with mild-to-moderate calcific valve disease (stages B to D) (54–56). (Level of Evidence: A)

3.4 Timing of Intervention

See Table 7 for a summary of recommendations from this section.

• 1. AVR is recommended in symptomatic patients with severe AS (stage D1) with (10,57–59) (Level of Evidence: B):

  • a. Decreased systolic opening of a calcified or congenitally stenotic aortic valve; and

  • b. An aortic velocity 4.0 m per second or greater or mean pressure gradient 40 mm Hg or higher; and

  • c. Symptoms of HF, syncope, exertional dyspnea, angina, or presyncope by history or on exercise testing.

• 2. AVR is recommended for asymptomatic patients with severe AS (stage C2) and an LVEF less than 50% with decreased systolic opening of a calcified aortic valve with an aortic velocity 4.0 m per second or greater or mean pressure gradient 40 mm Hg or higher (60,61). (Level of Evidence: B)

• 3. AVR is indicated for patients with severe AS (stage C or D) when undergoing cardiac surgery for other indications when there is decreased systolic opening of a calcified aortic valve and an aortic velocity 4.0 m per second or greater or mean pressure gradient 40 mm Hg or higher (62,63). (Level of Evidence: B)

• 1. AVR is reasonable for asymptomatic patients with very severe AS (stage C1) with (64,65) (Level of Evidence: B):

  • a. Decreased systolic opening of a calcified valve;

  • b. An aortic velocity 5.0 m per second or greater or mean pressure gradient 60 mm Hg or higher; and

  • c. A low surgical risk.

• 2. AVR is reasonable in apparently asymptomatic patients with severe AS (stage C1) with (27,38) (Level of Evidence: B):

  • a. A calcified aortic valve;

  • b. An aortic velocity of 4.0 m per second to 4.9 m per second or mean pressure gradient of 40 mm Hg to 59 mm Hg; and

  • c. An exercise test demonstrating decreased exercise tolerance or a fall in systolic blood pressure (BP).

• 3. AVR is reasonable in symptomatic patients with low-flow/low-gradient severe AS with reduced LVEF (stage D2) with a (66–68) (Level of Evidence: B):

  • a. Calcified aortic valve with reduced systolic opening;

  • b. Resting valve area 1.0 cm² or less;

  • c. Aortic velocity less than 4.0 m per second or mean pressure gradient less than 40 mm Hg;

  • d. LVEF less than 50%; and

  • e. A low-dose dobutamine stress study that shows an aortic velocity 4.0 m per second or greater or mean pressure gradient 40 mm Hg or higher with a valve area 1.0 cm² or less at any dobutamine dose.

• 4. AVR is reasonable in symptomatic patients with low-flow/low-gradient severe AS (stage D3) with an LVEF 50% or greater, a calcified aortic valve with significantly reduced leaflet motion, and a valve area 1.0 cm² or less only if clinical, hemodynamic, and anatomic data support valve obstruction as the most likely cause of symptoms and data recorded when the patient is normotensive (systolic BP <140 mm Hg) indicate (Level of Evidence: C):

  • a. An aortic velocity less than 4.0 m per second or mean pressure gradient less than 40 mm Hg; and

  • b. A stroke volume index less than 35 mL/m²; and

  • c. An indexed valve area 0.6 cm²/m² or less.

• 5. AVR is reasonable for patients with moderate AS (stage B) with an aortic velocity between 3.0 m per second and 3.9 m per second or mean pressure gradient between 20 mm Hg and 39 mm Hg who are undergoing cardiac surgery for other indications. (Level of Evidence: C)

• 1. AVR may be considered for asymptomatic patients with severe AS (stage C1) with an aortic velocity 4.0 m per second or greater or mean pressure gradient 40 mm Hg or higher if the patient is at low surgical risk and serial testing shows an increase in aortic velocity 0.3 m/s or greater per year. (Level of Evidence: C)

3.5 Choice of Intervention

See Table 8 for a summary of recommendations from this section.

• 1. Surgical AVR is recommended in patients who meet an indication for AVR (Section 3.4) with low or intermediate surgical risk (Section 2.5 in the full-text guideline) (69,70). (Level of Evidence: A)

• 2. For patients in whom TAVR or high-risk surgical AVR is being considered, a Heart Valve Team consisting of an integrated, multidisciplinary group of healthcare professionals with expertise in VHD, cardiac imaging, interventional cardiology, cardiac anesthesia, and cardiac surgery should collaborate to provide optimal patient care. (Level of Evidence: C)

• 3. TAVR is recommended in patients who meet an indication for AVR (Section 3.4) who have a prohibitive risk for surgical AVR (Section 2.5 in the full-text guideline) and a predicted post-TAVR survival greater than 12 months (71,72). (Level of Evidence: B)

• 1. TAVR is a reasonable alternative to surgical AVR in patients who meet an indication for AVR (Section 3.4) and who have high surgical risk for surgical AVR (Section 2.5 in the full-text guideline) (73,74). (Level of Evidence: B)

• 1. Percutaneous aortic balloon dilation may be considered as a bridge to surgical AVR or TAVR in patients with severe symptomatic AS. (Level of Evidence: C)

• 1. TAVR is not recommended in patients in whom existing comorbidities would preclude the expected benefit from correction of AS (71). (Level of Evidence: B)

4.1 Stages of Chronic Aortic Regurgitation

The most common causes of chronic aortic regurgitation (AR) in the United States and other developed countries are bicuspid aortic valve and calcific valve disease. In addition, AR frequently arises from primary diseases causing dilation of the ascending aorta or the sinuses of Valsalva. Another cause of AR is rheumatic heart disease (the leading cause in many developing countries). In the majority of patients with AR, the disease course is chronic and slowly progressive with increasing LV volume overload and LV adaptation via chamber dilation and hypertrophy. Management of patients with AR depends on accurate diagnosis of the cause and stage of the disease process. Table 9⇓ shows the stages of AR ranging from patients at risk of AR (stage A) or with progressive mild-to-moderate AR (stage B) to severe asymptomatic (stage C) and symptomatic AR (stage D). Each of these stages is defined by valve anatomy, valve hemodynamics, severity of LV dilation, and LV systolic function, as well as by patient symptoms.

See Figure 2 for indications for AVR for chronic AR.

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Figure 2

Indications for AVR for Chronic AR

AR indicates aortic regurgitation; AVR, aortic valve replacement (valve repair may be appropriate in selected patients); ERO, effective regurgitant orifice; LV, left ventricular; LVEDD, left ventricular end-diastolic dimension; LVEF, left ventricular ejection fraction; LVESD, left ventricular end-systolic dimension; RF, regurgitant fraction; and RVol, regurgitant volume.

4.2 Diagnosis and Follow-Up

• 1. TTE is indicated in patients with signs or symptoms of AR (stages A to D) for accurate diagnosis of the cause of regurgitation, regurgitant severity, and LV size and systolic function, and for determining clinical outcome and timing of valve intervention (34,75–84). (Level of Evidence: B)

• 2. TTE is indicated in patients with dilated aortic sinuses or ascending aorta or with a bicuspid aortic valve (stages A and B) to evaluate the presence and severity of AR (85). (Level of Evidence: B)

• 3. CMR is indicated in patients with moderate or severe AR (stages B, C, and D) and suboptimal echocardiographic images for the assessment of LV systolic function, systolic and diastolic volumes, and measurement of AR severity (86,87). (Level of Evidence: B)

4.3 Medical Therapy

• 1. Treatment of hypertension (systolic BP >140 mm Hg) is recommended in patients with chronic AR (stages B and C), preferably with dihydropyridine calcium channel blockers or angiotensin-converting enzyme (ACE) inhibitors/angiotensin-receptor blockers (ARBs) (83,88). (Level of Evidence: B)

• 1. Medical therapy with ACE inhibitors/ARBs and beta blockers is reasonable in patients with severe AR who have symptoms and/or LV dysfunction (stages C2 and D) when surgery is not performed because of comorbidities (89,90). (Level of Evidence: B)

4.4 Timing of Intervention

See Table 10 for a summary of recommendations from this section.

• 1. AVR is indicated for symptomatic patients with severe AR regardless of LV systolic function (stage D) (33,91,92). (Level of Evidence: B)

• 2. AVR is indicated for asymptomatic patients with chronic severe AR and LV systolic dysfunction (LVEF <50%) at rest (stage C2) if no other cause for systolic dysfunction is identified (91,93–95). (Level of Evidence: B)

• 3. AVR is indicated for patients with severe AR (stage C or D) while undergoing cardiac surgery for other indications. (Level of Evidence: C)

• 1. AVR is reasonable for asymptomatic patients with severe AR with normal LV systolic function (LVEF ≥50%) but with severe LV dilation (LV end-systolic dimension [LVESD] >50 mm or indexed LVESD >25 mm/m²) (stage C2) (96–98). (Level of Evidence: B)

• 2. AVR is reasonable in patients with moderate AR (stage B) while undergoing surgery on the ascending aorta, coronary artery bypass graft (CABG), or mitral valve surgery. (Level of Evidence: C)

• 1. AVR may be considered for asymptomatic patients with severe AR and normal LV systolic function at rest (LVEF ≥50%, stage C1) but with progressive severe LV dilatation (LV end-diastolic dimension >65 mm) if surgical risk is low. (Level of Evidence: C)

5.1 Diagnosis and Follow-Up

• 1. An initial TTE is indicated in patients with a known bicuspid aortic valve to evaluate valve morphology, to measure the severity of AS and AR, and to assess the shape and diameter of the aortic sinuses and ascending aorta for prediction of clinical outcome and to determine timing of intervention (99–104). (Level of Evidence: B)

• 2. Aortic magnetic resonance angiography or CT angiography is indicated in patients with a bicuspid aortic valve when morphology of the aortic sinuses, sinotubular junction, or ascending aorta cannot be assessed accurately or fully by echocardiography. (Level of Evidence: C)

• 3. Serial evaluation of the size and morphology of the aortic sinuses and ascending aorta by echocardiography, CMR, or CT angiography is recommended in patients with a bicuspid aortic valve and an aortic diameter greater than 4.0 cm, with the examination interval determined by the degree and rate of progression of aortic dilation and by family history. In patients with an aortic diameter greater than 4.5 cm, this evaluation should be performed annually. (Level of Evidence: C)

5.2 Intervention

• 1. Operative intervention to repair the aortic sinuses or replace the ascending aorta is indicated in patients with a bicuspid aortic valve if the diameter of the aortic sinuses or ascending aorta is greater than 5.5 cm (105–107). (Level of Evidence: B)

• 1. Operative intervention to repair the aortic sinuses or replace the ascending aorta is reasonable in patients with bicuspid aortic valves if the diameter of the aortic sinuses or ascending aorta is greater than 5.0 cm and a risk factor for dissection is present (family history of aortic dissection or if the rate of increase in diameter is ≥0.5 cm per year). (Level of Evidence: C)

• 2. Replacement of the ascending aorta is reasonable in patients with a bicuspid aortic valve who are undergoing aortic valve surgery because of severe AS or AR (Sections 3.4 and 4.4) if the diameter of the ascending aorta is greater than 4.5 cm. (Level of Evidence: C)

6.1 Stages of MS

Medical and interventional approaches to the management of patients with valvular MS depend on accurate diagnosis of the cause and stage of the disease process. Table 11⇓ shows the stages of mitral valve disease ranging from patients at risk of MS (stage A) or with progressive hemodynamic obstruction (stage B) to severe asymptomatic (stage C) and symptomatic MS (stage D). Each of these stages is defined by valve anatomy, valve hemodynamics, the consequences of valve obstruction on the left atrium (LA) and pulmonary circulation, and patient symptoms. The anatomic features of the stages of MS are based on a rheumatic etiology for the disease. There are patients who have a nonrheumatic etiology of MS due to senile calcific disease (Section 6.3 in the full text) in whom there is a heavily calcified mitral annulus with extension of the calcium into the leaflets. Hemodynamic severity is best characterized by the planimetered mitral valve area and the calculated mitral valve area from the diastolic pressure half-time. The definition of “severe” MS is based on the severity at which symptoms occur as well as the severity at which intervention will improve symptoms. Thus, a mitral valve area ≤1.5 cm² is considered severe. This usually corresponds to a transmitral mean gradient of >5 mm Hg to 10 mm Hg at a normal heart rate. However, the mean pressure gradient is highly dependent on the transvalvular flow and diastolic filling period and will vary greatly with changes in heart rate. The diastolic pressure half-time is dependent not only on the degree of mitral obstruction but also the compliance of the left ventricle and LA and other measures of mitral valve area, such as the continuity equation or the proximal isovelocity surface area, may be used if discrepancies exist.

Supporting References: (108–114)

See Figure 3 for indications for intervention for rheumatic MS.

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Figure 3

Indications for Intervention for Rheumatic MS

AF indicates atrial fibrillation; LA, left atrial; MR, mitral regurgitation; MS, mitral stenosis; MVA, mitral valve area; MVR, mitral valve surgery (repair or replacement); NYHA, New York Heart Association; PCWP, pulmonary capillary wedge pressure; PMBC, percutaneous mitral balloon commissurotomy; and T ½, pressure half-time.

6.2 Diagnosis and Follow-Up

• 1. TTE is indicated in patients with signs or symptoms of MS to establish the diagnosis, quantify hemodynamic severity (mean pressure gradient, mitral valve area, and pulmonary artery pressure), assess concomitant valvular lesions, and demonstrate valve morphology (to determine suitability for mitral commissurotomy) (9,115–123). (Level of Evidence: B)

• 2. TEE should be performed in patients considered for percutaneous mitral balloon commissurotomy to assess the presence or absence of left atrial thrombus and to further evaluate the severity of mitral regurgitation (MR) (116,124–126). (Level of Evidence: B)

• 3. Exercise testing with Doppler or invasive hemodynamic assessment is recommended to evaluate the response of the mean mitral gradient and pulmonary artery pressure in patients with MS when there is a discrepancy between resting Doppler echocardiographic findings and clinical symptoms or signs. (Level of Evidence: C)

6.3 Medical Therapy

• 1. Anticoagulation (vitamin K antagonist [VKA] or heparin) is indicated in patients with 1) MS and AF (paroxysmal, persistent, or permanent), 2) MS and a prior embolic event, or 3) MS and a left atrial thrombus (127–133). (Level of Evidence: B)

• 1. Heart rate control can be beneficial in patients with MS and AF and fast ventricular response. (Level of Evidence: C)

• 1. Heart rate control may be considered for patients with MS in normal sinus rhythm and symptoms associated with exercise (134,135). (Level of Evidence: B)

6.4 Intervention

See Table 12 for a summary of recommendations from this section.

• 1. Percutaneous mitral balloon commissurotomy is recommended for symptomatic patients with severe MS (mitral valve area ≤1.5 cm², stage D) and favorable valve morphology in the absence of left atrial thrombus or moderate-to-severe MR (108–112,114,136). (Level of Evidence: A)

• 2. Mitral valve surgery (repair, commissurotomy, or valve replacement) is indicated in severely symptomatic patients (NYHA class III to IV) with severe MS (mitral valve area ≤1.5 cm², stage D) who are not high risk for surgery and who are not candidates for or who have failed previous percutaneous mitral balloon commissurotomy (137–142). (Level of Evidence: B)

• 3. Concomitant mitral valve surgery is indicated for patients with severe MS (mitral valve area ≤1.5 cm², stage C or D) undergoing cardiac surgery for other indications. (Level of Evidence: C)

• 1. Percutaneous mitral balloon commissurotomy is reasonable for asymptomatic patients with very severe MS (mitral valve area ≤1.0 cm², stage C) and favorable valve morphology in the absence of left atrial thrombus or moderate-to-severe MR (121,143–145). (Level of Evidence: C)

• 2. Mitral valve surgery is reasonable for severely symptomatic patients (NYHA class III to IV) with severe MS (mitral valve area ≤1.5 cm², stage D), provided there are other operative indications (e.g., aortic valve disease, coronary artery disease (CAD), tricuspid regurgitation (TR), aortic aneurysm). (Level of Evidence: C)

• 1. Percutaneous mitral balloon commissurotomy may be considered for asymptomatic patients with severe MS (mitral valve area ≤1.5 cm², stage C) and valve morphology favorable for percutaneous mitral balloon commissurotomy in the absence of left atrial thrombus or moderate-to-severe MR who have new onset of AF. (Level of Evidence: C)

• 2. Percutaneous mitral balloon commissurotomy may be considered for symptomatic patients with mitral valve area greater than 1.5 cm² if there is evidence of hemodynamically significant MS based on pulmonary artery wedge pressure greater than 25 mm Hg or mean mitral valve gradient greater than 15 mm Hg during exercise. (Level of Evidence: C)

• 3. Percutaneous mitral balloon commissurotomy may be considered for severely symptomatic patients (NYHA class III to IV) with severe MS (mitral valve area ≤1.5 cm², stage D) who have a suboptimal valve anatomy and who are not candidates for surgery or at high risk for surgery. (Level of Evidence: C)

• 4. Concomitant mitral valve surgery may be considered for patients with moderate MS (mitral valve area 1.6 cm² to 2.0 cm²) undergoing cardiac surgery for other indications. (Level of Evidence: C)

• 5. Mitral valve surgery and excision of the left atrial appendage may be considered for patients with severe MS (mitral valve area ≤1.5 cm², stages C and D) who have had recurrent embolic events while receiving adequate anticoagulation. (Level of Evidence: C)

7.1 Stages of Chronic MR

In assessing the patient with chronic MR, it is critical to distinguish between chronic primary (degenerative) MR and chronic secondary (functional) MR, as these 2 conditions have more differences than similarities.

In chronic primary MR, the pathology of ≥1 of the components of the valve (leaflets, chordae tendineae, papillary muscles, annulus) causes valve incompetence with systolic regurgitation of blood from the left ventricle to the LA (Table 13). The most common cause of chronic primary MR in developed countries is mitral valve prolapse, which has a wide spectrum of etiology and presentation. Younger populations present with severe myxomatous degeneration with gross redundancy of both anterior and posterior leaflets and the chordal apparatus (Barlow's valve). Alternatively, older populations present with fibroelastic deficiency disease, in which lack of connective tissue leads to chordal rupture. The differentiation between these 2 etiologies has important implications for operative intervention. Other less common causes of chronic primary MR include IE, connective tissue disorders, rheumatic heart disease, cleft mitral valve, and radiation heart disease. If the subsequent volume overload of chronic primary MR is prolonged and severe, it causes myocardial damage, HF, and eventual death. Correction of the MR is curative. Thus, MR is “the disease.”

In chronic secondary MR, the mitral valve is usually normal (Table 14). Instead, severe LV dysfunction is caused either by CAD, related myocardial infarction (ischemic chronic secondary MR), or idiopathic myocardial disease (nonischemic chronic secondary MR). The abnormal and dilated left ventricle causes papillary muscle displacement, which in turn results in leaflet tethering with associated annular dilation that prevents coaptation. Because MR is only 1 component of the disease (severe LV dysfunction, coronary disease, or idiopathic myocardial disease are the others), restoration of mitral valve competence is not by itself curative; thus, the best therapy for chronic secondary MR is much less clear than it is for chronic primary MR. The data are limited, and there is greater difficulty in defining the severity of MR in patients with secondary MR than in those with primary MR. In patients with secondary MR, adverse outcomes are associated with a smaller calculated effective regurgitant orifice compared to primary MR due to multiple reasons. The MR will likely progress due to the associated progressive LV systolic dysfunction and adverse remodeling. In addition, there is an underestimation of effective regurgitant orifice area by the 2-dimensional echocardiography−derived flow convergence method due to the crescentic shape of the regurgitant orifice. There are the additional clinical effects of a smaller amount of regurgitation in the presence of compromised LV systolic function and baseline elevated filling pressures.

7.2 Chronic Primary MR

7.3 Chronic Secondary MR

7.3.3 Intervention

See Table 16 for a summary of recommendations for this section and Figure 4 for indications for surgery for MR.

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Figure 4

Indications for Surgery for MR

*Mitral valve repair is preferred over MVR when possible.

AF indicates atrial fibrillation; CAD, coronary artery disease; CRT, cardiac resynchronization therapy; ERO, effective regurgitant orifice; HF, heart failure; LV, left ventricular; LVEF, left ventricular ejection fraction; LVESD, left ventricular end-systolic dimension; MR, mitral regurgitation, MV, mitral valve; MVR, mitral valve replacement; NYHA, New York Heart Association; PASP, pulmonary artery systolic pressure; RF, regurgitant fraction; RVol, regurgitant volume; and Rx, therapy.

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Table 16

Summary of Recommendations for Chronic Severe Secondary MR

Class IIa

• 1. Mitral valve surgery is reasonable for patients with chronic severe secondary MR (stages C and D) who are undergoing CABG or AVR. (Level of Evidence: C)

Class IIb

• 1. Mitral valve repair or replacement may be considered for severely symptomatic patients (NYHA class III to IV) with chronic severe secondary MR (stage D) who have persistent symptoms despite optimal GDMT for HF (224–235). (Level of Evidence: B)

• 2. Mitral valve repair may be considered for patients with chronic moderate secondary MR (stage B) who are undergoing other cardiac surgery. (Level of Evidence: C)

8.1 Stages of TR

Trace-to-mild degrees of TR of no physiological consequence are commonly detected on TTE in subjects with anatomically normal valves. Primary disorders of the tricuspid apparatus that can lead to more significant degrees of TR include rheumatic disease, prolapse, congenital disease (Ebstein's), IE, radiation, carcinoid, blunt chest wall trauma, RV endomyocardial biopsy–related trauma, and intra-annular RV pacemaker or implantable cardioverter-defibrillator leads. Approximately 80% of cases of significant TR are functional in nature and related to tricuspid annular dilation and leaflet tethering in the setting of RV remodeling due to pressure and/or volume overload. The tricuspid annulus is a saddle-shaped ellipsoid that becomes planar and circular as it dilates in an anterior-posterior direction and will often not return to its normal size and configuration after relief of RV overload. Table 17 shows the stages (A through D) of primary and functional TR as defined for other valve lesions. Severe TR (stages C and D) is associated with poor prognosis independent of age, LV and RV function, and RV size. Patients with signs or symptoms of right HF would fit into the stage D category even if they do not meet other hemodynamic or morphological criteria.

Supporting Reference: (236)

8.2 Tricuspid Regurgitation

See Figure 5 for indications for surgery.

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Figure 5

Indications for Surgery

*See Table 17 for definition of stages. TA dilation is defined by >40 mm on TTE (>21 mm/m²) or >70 mm on direct intraoperative measurement.

LV indicates left ventricular; PHTN, pulmonary hypertension; RV, right ventricular; TA, tricuspid annular; TR, tricuspid regurgitation; TTE, transthoracic echocardiogram; TV, tricuspid valve; and TVR, tricuspid valve replacement.

8.3 Stages of Tricuspid Stenosis

See Table 18 for the stages of severe tricuspid stenosis (TS).

8.4 Tricuspid Stenosis

10.1 Evaluation and Selection of Prosthetic Valves

10.2 Antithrombotic Therapy for Prosthetic Valves

• 1. Anticoagulation with a VKA and international normalized ratio (INR) monitoring is recommended in patients with a mechanical prosthetic valve (261–263). (Level of Evidence: A)

• 2. Anticoagulation with a VKA to achieve an INR of 2.5 is recommended in patients with a mechanical AVR (bileaflet or current-generation single tilting disc) and no risk factors for thromboembolism (264–266). (Level of Evidence: B)

• 3. Anticoagulation with a VKA is indicated to achieve an INR of 3.0 in patients with a mechanical AVR and additional risk factors for thromboembolic events (AF, previous thromboembolism, LV dysfunction, or hypercoagulable conditions) or an older-generation mechanical AVR (such as ball-in-cage) (267). (Level of Evidence: B)

• 4. Anticoagulation with a VKA is indicated to achieve an INR of 3.0 in patients with a mechanical MVR (267,268). (Level of Evidence: B)

• 5. Aspirin 75 mg to 100 mg daily is recommended in addition to anticoagulation with a VKA in patients with a mechanical valve prosthesis (269,270). (Level of Evidence: A)

• 1. Aspirin 75 mg to 100 mg per day is reasonable in all patients with a bioprosthetic aortic or mitral valve (271–274). (Level of Evidence: B)

• 2. Anticoagulation with a VKA is reasonable for the first 3 months after bioprosthetic MVR or repair to achieve an INR of 2.5 (275). (Level of Evidence: C)

• 1. Anticoagulation, with a VKA, to achieve an INR of 2.5 may be reasonable for the first 3 months after bioprosthetic AVR (276). (Level of Evidence: B)

• 2. Clopidogrel 75 mg daily may be reasonable for the first 6 months after TAVR in addition to life-long aspirin 75 mg to 100 mg daily. (Level of Evidence: C)

• 1. Anticoagulant therapy with oral direct thrombin inhibitors or anti-Xa agents should not be used in patients with mechanical valve prostheses (277–279). (Level of Evidence: B)

10.3 Bridging Therapy for Prosthetic Valves

• 1. Continuation of VKA anticoagulation with a therapeutic INR is recommended in patients with mechanical heart valves undergoing minor procedures (such as dental extractions or cataract removal) where bleeding is easily controlled. (Level of Evidence: C)

• 2. Temporary interruption of VKA anticoagulation, without bridging agents while the INR is subtherapeutic, is recommended in patients with a bileaflet mechanical AVR and no other risk factors for thrombosis who are undergoing invasive or surgical procedures. (Level of Evidence: C)

• 3. Bridging anticoagulation with either intravenous unfractionated heparin (UFH) or subcutaneous low-molecular-weight heparin (LMWH) is recommended during the time interval when the INR is subtherapeutic preoperatively in patients who are undergoing invasive or surgical procedures with a 1) mechanical AVR and any thromboembolic risk factor, 2) older-generation mechanical AVR, or 3) mechanical MVR. (Level of Evidence: C)

• 1. Administration of fresh frozen plasma or prothrombin complex concentrate is reasonable in patients with mechanical valves receiving VKA therapy who require emergency noncardiac surgery or invasive procedures. (Level of Evidence: C)

10.4 Excessive Anticoagulation and Serious Bleeding With Prosthetic Valves

See Figure 6 for anticoagulation for prosthetic valves.

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Figure 6

Anticoagulation for Prosthetic Valves

Risk factors include AF, previous thromboembolism, LV dysfunction, hypercoagulable condition, and older-generation mechanical AVR.

AF indicates atrial fibrillation; ASA, aspirin; AVR, aortic valve replacement; INR, international normalized ratio; LMWH, low-molecular-weight heparin; MVR, mitral valve replacement; PO, by mouth; QD, every day; SC, subcutaneous; TAVR, transcatheter aortic valve replacement; UFH, unfractionated heparin; and VKA, vitamin K antagonist.

• 1. Administration of fresh frozen plasma or prothrombin complex concentrate is reasonable in patients with mechanical valves and uncontrollable bleeding who require reversal of anticoagulation (280,281). (Level of Evidence: B)

10.5 Prosthetic Valve Thrombosis

See Figure 7 for evaluation and management of suspected valve thrombosis.

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Figure 7

Evaluation and Management of Suspected Prosthetic Valve Thrombosis

*See full-text guideline for dosage recommendations.

CT indicates computed tomography; IV, intravenous; NYHA, New York Heart Association; Rx, therapy; TEE, transesophageal echocardiography; and TTE, transthoracic echocardiography.

10.6 Prosthetic Valve Stenosis

• 1. Repeat valve replacement is indicated for severe symptomatic prosthetic valve stenosis. (Level of Evidence: C)

10.7 Prosthetic Valve Regurgitation

• 1. Surgery is recommended for operable patients with mechanical heart valves with intractable hemolysis or HF due to severe prosthetic or paraprosthetic regurgitation (291,292). (Level of Evidence: B)

• 1. Surgery is reasonable for operable patients with severe symptomatic or asymptomatic bioprosthetic regurgitation. (Level of Evidence C)

• 2. Percutaneous repair of paravalvular regurgitation is reasonable in patients with prosthetic heart valves and intractable hemolysis or NYHA class III/IV HF who are at high risk for surgery and have anatomic features suitable for catheter-based therapy when performed in centers with expertise in the procedure (293–295). (Level of Evidence B)

11.1 Diagnosis and Follow-Up

See Figure 8 for recommendations for imaging studies in native valve endocarditis and prosthetic valve endocarditis.

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Figure 8

Recommendations for Imaging Studies in NVE and PVE

*Repeat TEE and/or TTE recommended for reevaluation of patients with IE and a change in clinical signs or symptoms and in patients at high risk of complications.

CT indicates computed tomography; IE, infective endocarditis; NVE, native valve endocarditis; PVE, prosthetic valve endocarditis; S. aureus, Staphylococcus aureus; TEE, transesophageal echocardiography; and TTE, transthoracic echocardiography.

• 1. At least 2 sets of blood cultures should be obtained in patients at risk for IE (e.g., those with congenital or acquired VHD, previous IE, prosthetic heart valves, certain congenital or heritable heart malformations, immunodeficiency states, injection drug users) who have unexplained fever for more than 48 hours (296) (Level of Evidence: B) or patients with newly diagnosed left-sided valve regurgitation. (Level of Evidence: C)

• 2. The Modified Duke Criteria should be used in evaluating a patient with suspected IE (Tables 24 and 25 in the full-text guideline) (297–300). (Level of Evidence: B)

• 3. Patients with IE should be evaluated and managed with consultation of a multispecialty Heart Valve Team including an infectious disease specialist, cardiologist, and cardiac surgeon. In surgically managed patients, this team should also include a cardiac anesthesiologist (301). (Level of Evidence: B)

• 4. TTE is recommended in patients with suspected IE to identify vegetations, characterize the hemodynamic severity of valvular lesions, assess ventricular function and pulmonary pressures, and detect complications (302–306). (Level of Evidence: B)

• 5. TEE is recommended in all patients with known or suspected IE when TTE is nondiagnostic, when complications have developed or are clinically suspected, or when intracardiac device leads are present (307–315). (Level of Evidence: B)

• 6. TTE and/or TEE are recommended for re-evaluation of patients with IE who have a change in clinical signs or symptoms (e.g., new murmur, embolism, persistent fever, HF, abscess, or atrioventricular heart block) and in patients at high risk of complications (e.g., extensive infected tissue/large vegetation on initial echocardiogram or staphylococcal, enterococcal, fungal infections) (316,317). (Level of Evidence: B)

• 7. Intraoperative TEE is recommended for patients undergoing valve surgery for IE (318,319). (Level of Evidence: B)

• 1. TEE is reasonable to diagnose possible IE in patients with Staphylococcal aureus bacteremia without a known source (320–322). (Level of Evidence: B)

• 2. TEE is reasonable to diagnose IE of a prosthetic valve in the presence of persistent fever without bacteremia or a new murmur (323,324). (Level of Evidence: B)

• 3. Cardiac CT is reasonable to evaluate morphology/anatomy in the setting of suspected paravalvular infections when the anatomy cannot be clearly delineated by echocardiography (325–328). (Level of Evidence: B)

• 1. TEE might be considered to detect concomitant staphylococcal IE in nosocomial Staphylococcal aureus bacteremia with a known portal of entry from an extracardiac source (329–331). (Level of Evidence: B)

11.2 Medical Therapy

• 1. Appropriate antibiotic therapy should be initiated and continued after blood cultures are obtained with guidance from antibiotic sensitivity data and infectious disease consultants (296). (Level of Evidence: B)

• 1. It is reasonable to temporarily discontinue anticoagulation in patients with IE who develop central nervous system symptoms compatible with embolism or stroke regardless of the other indications for anticoagulation (332–337). (Level of Evidence: B)

• 1. Temporary discontinuation of VKA anticoagulation might be considered in patients receiving VKA anticoagulation at the time of IE diagnosis (333,338–341). (Level of Evidence: B)

• 1. Patients with known VHD should not receive antibiotics before blood cultures are obtained for unexplained fever. (Level of Evidence: C)

11.3 Intervention

See Figure 9 for diagnosis and treatment of IE.

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Figure 9

Diagnosis and Treatment of IE

*Early surgery defined as during initial hospitalization before completion of a full therapeutic course of antibiotics.

HF indicates heart failure; ICD, implantable cardioverter-defibrillator; IE, infective endocarditis; NVE, native valve endocarditis; PVE, prosthetic valve endocarditis; Rx, therapy; S. aureus, Staphylococcus aureus; TEE, transesophageal echocardiography; TTE, transthoracic echocardiography; and VKA, vitamin K antagonist.

• 1. Decisions about timing of surgical intervention should be made by a multispecialty Heart Valve Team of cardiology, cardiothoracic surgery, and infectious disease specialists (301). (Level of Evidence: B)

• 2. Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) is indicated in patients with IE who present with valve dysfunction resulting in symptoms of HF (342–347). (Level of Evidence: B)

• 3. Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) is indicated in patients with left-sided IE caused by Staphylococcal aureus, fungal, or other highly resistant organisms (347–354). (Level of Evidence: B)

• 4. Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) is indicated in patients with IE complicated by heart block, annular or aortic abscess, or destructive penetrating lesions (347,355–359). (Level of Evidence: B)

• 5. Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) for IE is indicated in patients with evidence of persistent infection as manifested by persistent bacteremia or fevers lasting longer than 5 to 7 days after onset of appropriate antimicrobial therapy (347,352,353,360–362). (Level of Evidence: B)

• 6. Surgery is recommended for patients with prosthetic valve endocarditis and relapsing infection (defined as recurrence of bacteremia after a complete course of appropriate antibiotics and subsequently negative blood cultures) without other identifiable source for portal of infection. (Level of Evidence: C)

• 7. Complete removal of pacemaker or defibrillator systems, including all leads and the generator, is indicated as part of the early management plan in patients with IE with documented infection of the device or leads (363–366). (Level of Evidence: B)

• 1. Complete removal of pacemaker or defibrillator systems, including all leads and the generator, is reasonable in patients with valvular IE caused by Staphylococcal aureus or fungi, even without evidence of device or lead infection (363–366). (Level of Evidence: B)

• 2. Complete removal of pacemaker or defibrillator systems, including all leads and the generator, is reasonable in patients undergoing valve surgery for valvular IE. (Level of Evidence: C)

• 3. Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) is reasonable in patients with IE who present with recurrent emboli and persistent vegetations despite appropriate antibiotic therapy (302,367,368). (Level of Evidence: B)

• 1. Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) may be considered in patients with native valve endocarditis who exhibit mobile vegetations greater than 10 mm in length (with or without clinical evidence of embolic phenomenon) (302,367,368). (Level of Evidence: B)

12.1 Native Valve Stenosis

• 1. All patients with suspected valve stenosis should undergo a clinical evaluation and TTE before pregnancy. (Level of Evidence: C)

• 2. All patients with severe valve stenosis (stages C and D) should undergo prepregnancy counseling by a cardiologist with expertise in managing patients with VHD during pregnancy. (Level of Evidence: C)

• 3. All patients referred for a valve operation before pregnancy should receive prepregnancy counseling by a cardiologist with expertise in managing patients with VHD during pregnancy about the risks and benefits of all options for operative interventions, including mechanical prosthesis, bioprosthesis, and valve repair. (Level of Evidence: C)

• 4. Pregnant patients with severe valve stenosis (stages C and D) should be monitored in a tertiary care center with a dedicated Heart Valve Team of cardiologists, surgeons, anesthesiologists, and obstetricians with expertise in the management of high-risk cardiac patients during pregnancy. (Level of Evidence: C)

12.2 Native Valve Regurgitation

12.3 Prosthetic Valves in Pregnancy

12.3.2 Medical Therapy

See Figure 10 for anticoagulation of pregnant patients with mechanical valves.

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Figure 10

Anticoagulation of Pregnant Patients With Mechanical Valves

aPTT indicates activated partial thromboplastin time; ASA, aspirin; INR, international normalized ratio; LMWH, low-molecular-weight heparin; QD, once daily; and UFH, unfractionated heparin.

Class I

• 1. Therapeutic anticoagulation with frequent monitoring is recommended for all pregnant patients with a mechanical prosthesis (384,385). (Level of Evidence: B)

• 2. Warfarin is recommended in pregnant patients with a mechanical prosthesis to achieve a therapeutic INR in the second and third trimesters (386–391). (Level of Evidence: B)

• 3. Discontinuation of warfarin with initiation of intravenous UFH (with an activated partial thromboplastin time [aPTT] >2 times control) is recommended before planned vaginal delivery in pregnant patients with a mechanical prosthesis. (Level of Evidence: C)

• 4. Low-dose aspirin (75 mg to 100 mg) once per day is recommended for pregnant patients in the second and third trimesters with either a mechanical prosthesis or bioprosthesis. (Level of Evidence: C)

Class IIa

• 1. Continuation of warfarin during the first trimester is reasonable for pregnant patients with a mechanical prosthesis if the dose of warfarin to achieve a therapeutic INR is 5 mg per day or less after full discussion with the patient about risks and benefits (384,385,390–393). (Level of Evidence: B)

• 2. Dose-adjusted LMWH at least 2 times per day (with a target anti-Xa level of 0.8 U/mL to 1.2 U/mL, 4 to 6 hours postdose) during the first trimester is reasonable for pregnant patients with a mechanical prosthesis if the dose of warfarin is greater than 5 mg per day to achieve a therapeutic INR (386–389,394,395). (Level of Evidence: B)

• 3. Dose-adjusted continuous intravenous UFH (with an aPTT at least 2 times control) during the first trimester is reasonable for pregnant patients with a mechanical prosthesis if the dose of warfarin is greater than 5 mg per day to achieve a therapeutic INR (384,385,392). (Level of Evidence: B)

Class IIb

• 1. Dose-adjusted LMWH at least 2 times per day (with a target anti-Xa level of 0.8 U/mL to 1.2 U/mL, 4 to 6 hours postdose) during the first trimester may be reasonable for pregnant patients with a mechanical prosthesis if the dose of warfarin is 5 mg per day or less to achieve a therapeutic INR (386–389,394–396). (Level of Evidence: B)

• 2. Dose-adjusted continuous infusion of UFH (with aPTT at least 2 times control) during the first trimester may be reasonable for pregnant patients with a mechanical prosthesis if the dose of warfarin is 5 mg per day or less to achieve a therapeutic INR (384,385,392). (Level of Evidence: B)

Class III: Harm

• 1. LMWH should not be administered to pregnant patients with mechanical prostheses unless anti-Xa levels are monitored 4 to 6 hours after administration (387,388,394,395,397). (Level of Evidence: B)

13.1 Evaluation of Coronary Anatomy

See Figure 11 for evaluation and management of CAD in patients undergoing valve surgery.

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Figure 11

Evaluation and Management of CAD in Patients Undergoing Valve Surgery

CABG indicates coronary artery bypass graft; CAD, coronary artery disease; CT, computed tomography; IE, infective endocarditis; LV, left ventricular; and PCI, percutaneous coronary intervention.

• 1. Coronary angiography is indicated before valve intervention in patients with symptoms of angina, objective evidence of ischemia, decreased LV systolic function, history of CAD, or coronary risk factors (including men age >40 years and postmenopausal women). (Level of Evidence: C)

• 2. Coronary angiography should be performed as part of the evaluation of patients with chronic severe secondary MR. (Level of Evidence: C)

• 1. Surgery without coronary angiography is reasonable for patients having emergency valve surgery for acute valve regurgitation, disease of the aortic sinuses or ascending aorta, or IE. (Level of Evidence: C)

• 2. CT coronary angiography is reasonable to exclude the presence of significant obstructive CAD in selected patients with a low/intermediate pretest probability of CAD. A positive coronary CT angiogram (the presence of any epicardial CAD) can be confirmed with invasive coronary angiography (398–404). (Level of Evidence: B)

13.2 Concomitant Procedures