ACC/AHA Guidelines and Treatment Options for Mitral Valve Disease


LEARNING OBJECTIVES

To review treatment options and mitral valve recommendations from the 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease and discuss differences compared to the 2021 European Society of Cardiology/European Association for Cardio-Thoracic Surgery (ESC/EACTS) Guidelines for the Management of Valvular Heart Disease.

INTRODUCTION

Guidelines and recommendations for the treatment of valvular syndromes are periodically published by the American College of Cardiology (ACC) and the American Heart Association (AHA). In this chapter we will review the mitral valve (MV) recommendations from the 2020 ACC/AHA Guideline for the Management of Patients with Valvular Heart Disease and discuss differences compared to the 2021 European Society of Cardiology/European Association for Cardio-Thoracic Surgery (ESC/EACTS) Guidelines for the Management of Valvular Heart Disease.

ACC/AHA strength of recommendations are assigned Class I (strong), Class IIa (moderate), Class IIb (weak), Class III (no benefit), and Class IIIb (harm), whereas in the ESC the divisions are worded slightly differently: Class I (recommended), Class IIa (should be considered), Class IIb (may be considered), and Class III (is not recommended). The ACC/AHA Levels of Evidence are categorized as A (high quality), Level B-R (moderate quality), Level B-NR (nonrandomized moderate quality), Level C-LD (limited data), and Level C-EO (expert opinion), whereas in the ESC they are again slightly different, with Level A (multiple randomized control trials [RCTs]), Level B (single RCT or large nonrandomized studies), and Level C (expert opinion or small studies).

Criteria for determining severity of, and recommendations for, MV syndromes are discussed in a different chapter and include additional factors aside from valve function, such as comorbidities, surgical risk, and cardiac and extra-cardiac end-organ damage. Accordingly, guidelines will not only involve active surgical or interventional treatments, but also include conservative management, watchful waiting, periodic monitoring, and postprocedural care. Therefore, guidelines stress thorough initial and subsequent evaluations ought to be performed in order to continuously optimize and individualize treatment. Evaluation should encompass establishing the stage of the disease ( Table 3.1 ), which takes into account symptomatology, physical examination, anatomy, severity, response of the ventricle and pulmonary circulation, and results of noninvasive and invasive testing as indicated. Finally, candidates for interventions should be evaluated by a multidisciplinary heart valve team (MDT) (recommendation I, Level C-EO).

TABLE 3.1
Stages of Valvular Heart Disease
Stage Definition Description
A At risk Patients with risk factors for development of VHD
B Progressive Patients with progressive VHD (mild to moderate severity and asymptomatic)
C Asymptomatic severe Asymptomatic patients who have the criteria for severe VHD
CI: Asymptomatic patients with severe VHD in whom the LV or RV remains compensated
CII: Asymptomatic patients with severe VHD with decompensation of the LV or RV
D Symptomatic severe Patients who have developed symptoms as a result of VHD
LV , Left ventricle; RV , right ventricle; VHD , valvular heart disease.
(From Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol . 2021;77(4):e25–e197.)

In a patient who is a candidate for intervention, risks should be calculated and discussed (recommendation I, Level C-EO) using an outcome score. In the US, the Society of Thoracic Surgeons (STS) score is most commonly used, although the European System for Cardiac Operative Risk Evaluation (EuroSCORE) II can also be considered ( Table 3.2 ). A high surgical risk patient is, strictly speaking, defined as having an STS score above 8% for mortality. Other important determinations of surgical risk not necessarily accounted for in the surgical risk scores include two or more frailty indices of moderate-to-severe frailty (indices such as Katz Activities of Daily Living, and independence in ambulation), a major organ system compromise (cardiac or extra-cardiac) that is predicted not to improve postoperatively, or possible procedure-specific considerations, including the presence of tracheostomy, porcelain ascending aorta, chest malformation, arterial coronary graft adherent to posterior chest wall, prior sternotomy, severe chronic obstructive pulmonary disease (COPD), pulmonary hypertension, and hepatic dysfunction and radiation damage. Prohibitive surgical risk is defined as STS score above 50% for mortality or major morbidity, two or more frailty indices of moderate-to-severe frailty, three or more major organ system compromise that is predicted not to improve postoperatively, or severe procedure-specific impediments. Finally, multivalvular disease must be assessed, and indicators of futility, such as STS score for mortality over 15%, life expectancy of less than 1 year, and a poor candidate for rehabilitation, should also be considered.

TABLE 3.2
Risk Assessment for Surgical Valve Procedures
Criteria Low-Risk SAVR (Must Meet ALL Criteria in This Column) Low-Risk Surgical Mitral Valve Repair for Primary MR (Must Meet ALL Criteria in This Column) High-Risk Surgical (Any One Criterion in This Column) Prohibitive Surgical Risk (Any One Criterion in This Column)
STS-predicted risk of death a <3% AND <1% AND >8% OR Predicted risk of death or major morbidity (all-cause) >50% at 1 year OR
Frailty b None AND None AND ≥2 Indices (moderate to severe) OR ≥2 Indices (moderate to severe) OR
Cardiac or other major organ system compromise not to be improved postoperatively c None AND None AND 1–2 Organ systems OR ≥3 Organ systems OR
Procedure-specific impediment d None None Possible procedure-specific impediment Severe procedure-specific impediment

a Use of the STS predicted Risk of Mortality ( http://riskcalc.sts.org/stswebriskcalc/#/ ) to predict risk in a given institution with reasonable reliability is appropriate only if institutional outcomes are within 1 standard deviation of the STS average observed/expected mortality ration for the procedure in question. The FUROSCORE II risk calculator may also be considered for use and is available at http://www.euroscore.org/calc.html .

b Seven frailty indices: Katz Activities of Daily Living (independence in feeding, bathing, dressing, transferring, toileting, and urinary continence) plus independence in ambulation (no walking aid or assistance required, or completion of a 5-m walk in <6 seconds). Other scoring systems can be applied to calculate no, mild, or moderate-to-severe frailty.

c Examples of major organ system compromise include cardiac dysfunction (severe LV systolic or diastolic dysfunction or RV dysfunction, fixed pulmonary hypertension); kidney dysfunction (chronic kidney disease, stage 3 or worse); pulmonary dysfunction (FEV 1 , <50% or DLCO 2 <50% of predicted); central nervous system dysfunction (dementia, Alzheimer disease, Parkinson disease, cerebrovascular accident with persistent physical limitation); gastrointestinal dysfunction (Crohn disease, ulcerative colitis, nutritional impairment, or serum albumin <3.0); cancer (active malignancy); and liver dysfunction (any history of cirrhosis, variceal bleeding, or elevated INR in the absence of VKA therapy).

d Examples of procedure-specific impediments include presence of tracheostomy, heavily calcified (porcelain) ascending aorta, chest malformation, arterial coronary graft adherent to posterior chest wall, and radiation damage. DLCO 2 , Diffusion capacity for carbon dioxide; FEV 1 , forced expiratory volume in 1 second; INR , international normalized ratio; LV , left ventricular; MR , mitral regurgitation; RV , right ventricular; SAVR , surgical aortic valve replacement; STS , Society of Thoracic Surgeons; VKA , vitamin K antagonist. (From Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol . 2021;77(4):e25–e197.)

MITRAL REGURGITATION

Over the years, guidelines have had to accommodate the progression in therapeutic options as new technologies have emerged. Threshold for nonsurgical interventions is continuously lowered since there is less impact of traditional comorbidities on outcomes, and with greater technical expertise, better patient selection, and generational device improvements procedural complications have generally declined and clinical outcomes improved. Percutaneous therapies such as the U.S. Food and Drug Administration (FDA)-approved edge-to-edge repair, as well as current investigational valve implantation, annuloplasty, and technologies aiming at chordae repair strategies, have substantially impacted management of patients with mitral regurgitation (MR) in clinical practice. Aims of invasive treatment include alleviation of symptoms, interrupting the vicious cycle of left ventricular (LV) volume overload and its sequalae, and improving mortality outcomes.

MR can be divided into acute and chronic, and treatment options vary accordingly and may be influenced by the specific etiology of MR. A further mechanistic division, which is more relevant to the chronic MR, includes primary and secondary MR.

Acute Mitral Regurgitation

Whether acute MR results from leaflet perforation, chordal rupture, or rupture of the papillary muscle, acute LV volume overload, low forward flow, and pulmonary congestion ensue, along with the potential for acute hemodynamic decompensation. Initial management includes vasodilator therapy and intra-aortic balloon counter-pulsation (IABP) or other means of hemodynamic support. Both guidelines, ESC and ACA/AHA, suggest the possible use of diuretics; however, as there are no supporting data, firm recommendations were not issued. Undoubtedly, prompt MV surgery, preferably repair, is indicated when feasible, as it is lifesaving in many instances. Although surgery has been the traditional mode of management, there are emerging reports on successful urgent/emergent transcatheter interventions for severe acute MR.

Chronic Mitral Regurgitation

Imaging

As noted above, chronic MR can be divided into primary or degenerative, and secondary or functional MR. Generally speaking, degenerative MR results from primary MV pathologies (may be more than one component), while functional MR results from associated chamber pathology. Transthoracic echocardiography (TTE) is indicated for initial screening and evaluation of primary MR (recommendation I, Level B-NR) and is quite accurate in determining the severity of regurgitation, whereas transesophageal echocardiography (TEE) is indicated both when TTE provides insufficient or discordant information, and to better define the anatomic pathology in detail (recommendation I, Level C-EO). Cardiac magnetic resonance (CMR) imaging is indicated when there is a discrepancy in regurgitant severity between the findings on clinical assessment and echocardiography since CMR volume—and derivative regurgitant—determinations are quite accurate (recommendation I, Level B-NR). Intraoperative or intraprocedural TEE is indicated to provide guidance and to evaluate the degree of regurgitation at the end of surgical MV repair (MVr), as even mild MR is a risk factor for late failure ( Table 3.3 ). As with degenerative MR, TEE is indicated in advance of percutaneous repair of secondary chronic MR to determine suitability for the procedure ( Table 3.4 ).

TABLE 3.3
Diagnostic Testing: Initial Diagnosis
Recommendations for Diagnostic Testing: Initial Diagnosis of Chronic Mitral Regurgitation
COR LOE Recommendations
I B-NR
  • 1.

    In patients with known or suspected primary MR, TTE is indicated for baseline evaluation of LV size and function, RV function, LA size, pulmonary artery pressure, and the mechanism and severity of primary MR (stages A–D).

I C-EO
  • 2.

    In patients with primary MR, when TTE provides insufficient or discordant information, TEE is indicated for evaluation of the severity of MR, mechanism of MR, and status of LV function (stages B–D).

I B-NR
  • 3.

    In patients with primary MR, CMR is indicated to assess LV and RV volumes and function and may help with assessing MR severity when there is a discrepancy between the findings on clinical assessment and echocardiography.

I B-NR
  • 4.

    In patients with severe primary MR undergoing mitral intervention, intraoperative TEE is indicated to establish the anatomic basis for primary MR (stages C and D) and to guide repair.

CMR, Cardiac magnetic resonance; LA, left atrium/left atrial; LV, left ventricle; MR, mitral regurgitation; RV, right ventricle; TEE, transesophageal echocardiography; TTE, transthoracic echocardiography.
(From Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol . 2021;77(4):e25–e197.)

TABLE 3.4
Diagnosis of Chronic Secondary Mitral Regurgitation
Recommendations for Diagnosis of Secondary Mitral Regurgitation
COR LOE Recommendations
I B-NR
  • 1.

    In patients with chronic secondary MR (stages B–D), TTE is useful to establish the etiology and to assess the extent of regional and global LV remodeling and systolic dysfunction, severity of MR, and magnitude to pulmonary hypertension.

I C-EO
  • 2.

    In patients with chronic secondary MR (stages B–D), noninvasive imaging (stress nuclear/PET, CMR, or stress echocardiography), coronary CT angiography, or coronary arteriography is useful to establish etiology of MR to assess myocardial viability.

I B-NR
  • 3.

    In patients with chronic secondary MR with severe symptoms (stage D) that are unresponsive to GDMT who are being considered for transcatheter mitral valve interventions, TTE is indicated to determine suitability for the procedure.

I C-EO
  • 4.

    In patients with chronic secondary MR undergoing transcatheter mitral valve intervention, intraprocedural guidance with TEE is recommended.

CMR, Cardiac magnetic resonance; CT, computed tomography; GDMT, guideline-directed management and therapy; LV, left ventricle; MR, mitral regurgitation; PET, positron emission tomography; TEE, transesophageal echocardiography; TTE, transthoracic echocardiography.
(From Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2021;77(4):e25–e197.)

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