Valvular Heart Disease Aortic Stenosis onsultants: Rick A. Nishimura, MD, MA, FAHA o-hair Professor of Medicine Division of ardiovascular Disease Mayo linic atherine M. Otto, MD, FA, FAHA o-hair Professor of Medicine Division of ardiology University of Washington Key Points Evaluation Disease States Guidelineentral.com
Table of ontents Key Points Table 1. The Heart Valve Team Evaluation Table 2. nitial Diagnostic Testing Guidelineentral.com Table 3. Frequency of Echocardiograms in Asymptomatic Patients With VHD and Normal LV Function Table 4. Stages of Progression of VHD Table 5. Risk Assessment ombining STS Risk Estimate, Frailty, Major Organ System Dysfunction, and Procedure-Specific mpediments Treatment Table 6. Evaluation and Treatment in Patients With AS Table 7. ndications for AVR in Patients With AS Table 8. hoice of ntervention in Patients With AS Table 9. Stages of Valvular AS Figure 1. ndications for AVR in Patients With AS Recommendation Grading Table Abbreviations
Key Points Evaluation ack to Table of ontents ÎÎPatients with VHD may present with a heart murmur, symptoms, or incidental findings of valvular abnormalities on noninvasive testing. ÎÎThe initial evaluation should include a detailed history and physical exam, EG, chest x-ray, and transthoracic echocardiogram. ÎÎDecisions about treatment are based primarily on the presence or absence of symptoms, severity of VHD, and response of the ventricle to pressure or volume overload imposed by VHD. ÎÎVHD requires a multidisciplinary team and approach for its diagnosis and management. ÎÎReasons for valve intervention are to improve symptoms, prolong survival, and reduce the risk of complications. ÎÎAn evaluation of the surgical or interventional risk for each individual patient should be performed if intervention is indicated which includes a standard surgical risk score, along with consideration of comorbidites, frailty, and procedure specfic impediments. ÎÎFollow-up of patients with VHD is important to assess symptom status, provide patient education, and monitor disease severity, typically with periodic echocardiography. Table 2. nitial Diagnostic Testing Recommendations LOE 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. TTE is recommended in patients with known VHD with any change in symptoms or physical examination findings. Periodic monitoring with TTE is recommended in asymptomatic patients with known VHD at intervals depending on valve lesion, severity, ventricular size, and ventricular function. ardiac 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. Exercise testing is reasonable in selected patients with asymptomatic severe VHD to confirm the absence of symptoms, or assess the hemodynamic response to exercise, or determine prognosis. a The Heart Valve Team Table 1. The Heart Valve Team Recommendations LOE Patients with severe AS should be evaluated by a multidisciplinary Heart Valve Team when intervention is considered. onsultation with or referral to a Heart Valve enter of Excellence is reasonable when discussing treatment options for asymptomatic patients with severe AS, patients who may benefit from valve repair versus valve replacement, or patients with multiple comorbidities for whom valve intervention is considered. a Table 3. Frequency of Echocardiograms in Asymptomatic Patients With VHD and Normal LV Function Valve Lesion Stage AS a AR MS MR Progressive (stage ) Severe (stage ) Every 3-5 y (mild severity: V max 2.0-2.9 m/s) Every 1-2 y (moderate severity: V max 3.0-3.9 m/s) Every 6-12 mo (V max 4 m/s) Every 3-5 y (mild severity) Every 1-2 y (moderate severity) Every 6-12 mo Dilating LV: more frequently Every 3-5 y (MVA >1.5 cm 2 ) Every 3-5 y (mild severity) Every 1-2 y (moderate severity) Every 1-2 y Every 6-12 mo (MVA 1.0-1.5 cm 2 ) Dilating LV: Once every year more frequently (MVA <1.0 cm 2 ) Patients with mixed valve disease may require serial evaluations at intervals earlier than recommended for single valve lesions. a With normal stroke volume.
Evaluation Treatment ack to Table of ontents Table 4. Stages of Progression of VHD Stage Definition Description A At risk Patients with risk factors for development of VHD Progressive Patients with progressive VHD (mild-to-moderate severity and asymptomatic) D Table 5. Risk Assessment ombining STS Risk Estimate, Frailty, Major Organ System Dysfunction, and Procedure- Specific mpediments STS PROM a <4% AND Frailty b Asymptomatic severe Symptomatic severe Low Risk (Must Meet ALL riteria in This olumn) None AND Major organ None system AND compromise not to be improved postoperatively c ntermediate Risk (Any 1 riterion in This olumn) 4%-8% 1 index (mild) 1 organ system Procedurespecific None Possible impediment d impediment procedure-specific Asymptomatic patients who meet the criteria for severe VHD: 1: Asymptomatic patients with severe VHD in whom the left or right ventricle remains compensated 2: Asymptomatic patients with severe VHD, with decompensation of the left or right ventricle Patients who have developed symptoms as a result of VHD High Risk (Any 1 riterion in This olumn) >8% 2 indices (moderate to severe) No more than 2 organ systems Possible procedure-specific impediment Prohibitive Risk (Any 1 riterion in This olumn) Predicted risk with surgery of death or major morbidity (all-cause) >50% at 1 y 3 organ systems Severe procedurespecific impediment a Use of the STS PROM to predict risk in a given institution with reasonable reliability is appropriate only if institutional outcomes are within 1 standard deviation of STS average observed/expected ratio for the procedure in question. b Seven frailty indices: Katz Activities of Daily Living (independence in feeding, bathing, dressing, transferring, toileting, and urinary continence) and independence in ambulation (no walking aid or assist required or 5-meter walk in <6 s). Other scoring systems can be applied to calculate no, mild-, or moderate-to-severe frailty. c Examples of major organ system compromise: ardiac severe LV systolic or diastolic dysfunction or RV dysfunction, fixed PHTN; KD stage 3 or worse; pulmonary dysfunction with FEV1 <50% or DLO 2 <50% of predicted; NS dysfunction (dementia, Alzheimer s disease, Parkinson s disease, stroke with persistent physical limitation); G dysfunction rohn s disease, ulcerative colitis, nutritional impairment, or serum albumin <3.0; cancer active malignancy; and liver any history of cirrhosis, variceal bleeding, or elevated NR in the absence of VKA therapy. d Examples: tracheostomy present, heavily calcified ascending aorta, chest malformation, arterial coronary graft adherent to posterior chest wall, or radiation damage. Table 6. Evaluation and Treatment in Patients With AS Recommendations LOE Diagnostic Testing 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. Low-dose dobutamine stress testing using echocardiographic or invasive hemodynamic measurements is reasonable in patients with stage D2 AS with all of the following: alcified aortic valve with reduced systolic opening; LVEF <50%; alculated valve area 1.0 cm 2 ; and Aortic velocity <4.0 m/s or Pmean <40 mm Hg. 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 aortic velocity 4.0 m/s or Pmean 40 mm Hg (stage ). Exercise testing should NOT be performed in symptomatic patients with AS when the aortic velocity is 4.0 m/s or Pmean is 40 mm Hg (stage D). Medical Therapy Hypertension in patients at risk for developing AS (stage A) and in patients with asymptomatic AS (stages and ) should be treated according to standard GDMT, started at a low dose and gradually titrated upward as needed with frequent clinical monitoring. Vasodilator therapy may be reasonable if used with invasive hemodynamic monitoring in the acute management of patients with severe decompensated AS (stage D) with NYHA class V HF symptoms. Statin therapy is NOT indicated for prevention of hemodynamic progression of AS in patients with mild-to-moderate calcific valve disease (stages -D). a : Harm b : No enefit A
Treatment ack to Table of ontents Table 7. ndications for AVR in Patients With AS Recommendations LOE AVR is recommended in symptomatic patients with severe AS (stage D1) with: Decreased systolic opening of a calcified or congenitally stenotic aortic valve; and Aortic velocity 4.0 m/s or Pmean 40 mm Hg; and Symptoms of HF, syncope, exertional dyspnea, angina, or presyncope by history or on exercise testing. AVR is recommended for asymptomatic patients with severe AS (stage 2) and LVEF <50% with decreased systolic opening of a calcified aortic valve with an aortic velocity 4.0 m/s or Pmean 40 mm Hg. AVR is indicated for patients with very severe AS (stage or D) when undergoing cardiac surgery for other indications when there is decreased systolic opening of a calcified aortic valve and aortic velocity 4.0 m/s or Pmean 40 mm Hg. AVR is reasonable for asymptomatic patients with severe AS (stage 1) with: Decreased systolic opening of a calcified valve; Aortic velocity 5.0 m/s or Pmean 60 mm Hg; Low surgical risk. AVR is reasonable in apparently asymptomatic patients with severe AS (stage 1) with: A calcified aortic valve; Aortic velocity 4.0-4.9 m/s or Pmean of 40-59 mm Hg; An exercise test demonstrating decreased exercise tolerance or a fall in systolic P. AVR is reasonable in symptomatic patients with low-flow/lowgradient severe AS with reduced LVEF (stage D2) with a: alcified aortic valve with reduced systolic opening; Resting valve area 1.0 cm 2 ; Aortic velocity <4 m/s or Pmean<40 mm Hg; LVEF <50%; and Low-dose dobutamine stress study that shows aortic velocity 4 m/s or Pmean 40 mm Hg with valve area 1.0 cm 2 at any dobutamine dose. a a a Table 7. ndications for AVR in Patients With AS (cont'd) Recommendations LOE AVR is reasonable in symptomatic patients with low-flow/ low-gradient severe AS (stage D3) with LVEF 50%, a calcified aortic valve with significantly reduced leaflet motion, and a valve area 1.0 cm 2 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 P <140 mm Hg) indicate: An aortic velocity <4 m/s or Pmean <40 mm Hg; A stroke volume index <35 ml/m 2 ; and An indexed valve area 0.6 cm 2 /m 2. AVR is reasonable for patients with moderate AS (stage ) with an aortic velocity 3.0-3.9 m/s or Pmean 20-39 mm Hg who are undergoing cardiac surgery for other indications. AVR may be considered for asymptomatic patients with severe AS (stage 1) with aortic velocity 4.0 m/s or Pmean 40 mm Hg if the patient is at low surgical risk and serial testing shows an increase in aortic velocity 0.3 m/s/y. Table 8. hoice of ntervention in Patients With AS Recommendations LOE Surgical AVR is recommended in patients who meet an indication for AVR with low or intermediate surgical risk. 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. TAVR is recommended in patients who meet an indication for AVR who have a prohibitive risk for surgical AVR and a predicted post-tavr survival >12 months. TAVR is a reasonable alternative to surgical AVR in patients who meet an indication for AVR and who have high surgical risk for surgical AVR. Percutaneous aortic balloon dilation may be considered as a bridge to surgical AVR or TAVR in patients with severe symptomatic AS. TAVR is NOT recommended in patients in whom existing comorbidities would preclude the expected benefit from correction of AS. a a b a b : No enefit A
Treatment ack to Table of ontents Table 9. Stages of Valvular AS Stage Definition Valve Anatomy Valve Hemodynamics A At risk of AS icuspid aortic valve (or other congenital valve anomaly) Aortic valve sclerosis Progressive AS Mild-to-moderate leaflet calcification of a bicuspid or trileaflet valve with some reduction in systolic motion or Rheumatic valve changes with commissural fusion : Asymptomatic severe AS 1 Asymptomatic severe AS Severe leaflet calcification or congenital stenosis with severely reduced leaflet opening 2 Asymptomatic severe AS with LV dysfunction D: Symptomatic severe AS D1 Symptomatic severe highgradient AS Severe leaflet calcification or congenital stenosis with severely reduced leaflet opening Severe leaflet calcification or congenital stenosis with severely reduced leaflet opening Hemodynamic onsequences Aortic V max <2 m/s None None Mild AS: Aortic V max 2.0-2.9 m/s or Pmean <20 mm Hg Moderate AS: Aortic V max 3.0-3.9 m/s or Pmean 20-39 mm Hg Aortic V max 4 m/s or Pmean 40 mm Hg AVA typically 1.0 cm 2 (or AVAi 0.6 cm 2 /m 2 ) Very severe AS: Aortic V max 5 m/s or Pmean 60 mm Hg Aortic V max 4 m/s or Pmean 40 mm Hg AVA typically 1.0 cm 2 (or AVAi 0.6 cm 2 /m 2 ) Aortic V max 4 m/s or Pmean 40 mm Hg AVA typically 1.0 cm 2 (or AVAi 0.6 cm 2 /m 2 ) but may be larger with mixed AS/AR Early LV diastolic dysfunction may be present Normal LVEF LV diastolic dysfunction Mild LV hypertrophy Normal LVEF LVEF <50% LV diastolic dysfunction LV hypertrophy PHTN may be present Symptoms None None: Exercise testing is reasonable to confirm symptom status None Exertional dyspnea or decreased exercise tolerance Exertional angina Exertional syncope or presyncope D2 D3 Symptomatic severe low-flow/lowgradient AS with reduced LVEF Symptomatic severe low-gradient AS with normal LVEF or paradoxical low-flow severe AS Severe leaflet calcification with severely reduced leaflet motion Severe leaflet calcification with severely reduced leaflet motion AVA 1.0 cm 2 with resting aortic V max <4 m/s or Pmean <40 mm Hg DSE shows AVA 1.0 cm 2 with V max 4 m/s at any flow rate AVA 1.0 cm 2 with aortic V max <4 m/s or Pmean <40 mm Hg ndexed AVA 0.6 cm 2 /m 2 and Stroke volume index <35 ml/m 2 Measured when patient is normotensive (systolic P <140 mm Hg) LV diastolic dysfunction LV hypertrophy LVEF <50% ncreased LV relative wall thickness Small LV chamber with low stroke volume Restrictive diastolic filling LVEF 50% HF Angina Syncope or presyncope HF Angina Syncope or presyncope
Treatment Symptomatic (stage D1) AVR () Figure 1. ndications for AVR in Patients With AS Severe AS Vmax 4 m/s Pmean 40 mm Hg AVR (a) Abnormal Aortic Valve With Reduced Systolic Opening Asymptomatic (stage D1) LVEF <50% (stage 2) Other cardiac surgery Vmax 5 m/s Pmean 60 mm Hg Low surgical risk Abnormal ETT Vmax 0.3 m/s/y Low surgical risk AVR (b) Vmax 3-3.9 m/s Pmean 20-39 mm Hg Symptomatic LVEF <50% YES DSE with AVA 1 cm 2 and Vmax 4 m/s (stage D2) NO Asymptomatic (stage ) AVA 1 cm 2 and LVEF 50% (stage D3 a ) AS likely cause of symptoms AVR (a) Other cardiac surgery Arrows show decision pathways that result in a recommendation for AVR. Periodic monitoring is indicated for all patients in whom AVR is not yet indicated, including those with asymptomatic AS (stage D or ) and those with low-gradient AS (stage D2 or D3) who do not meet the criteria for intervention. a AVR should be considered with stage D3 AS only if valve obstruction is the most likely cause of symptoms, stroke volume index is <35 ml/m 2, indexed AVA is 0.6 cm 2 /m 2, and data are recorded when the patient is normotensive (systolic P <140 mm Hg). A recommendation with Level of Evidence or does not imply that the recommendation is weak. Many important clinical questions addressed in the guidelines do not lend themselves to clinical trials. Even when randomized trials are unavailable, there may be a very clear clinical consensus that a particular test or therapy is useful or effective. a Data available from clinical trials or registries about the usefulness/efficacy in different subpopulations, such as sex, age, history of diabetes, history of prior M, history of HF, and prior aspirin use. b For comparative effectiveness recommendations (lass and a; Level of Evidence A and only), studies that support the use of comparator verbs should involve direct comparisons of the treatments or strategies being evaluated. omparative effectiveness phrases b : treatment/strategy A is recommended/indicated in preference to treatment treatment A should be chosen over treatment treatment/strategy A is probably recommended/indicated in preference to treatment it is reasonable to choose treatment A over treatment Suggested phrases for writing recommendations: should is recommended is indicated is /beneficial is reasonable can be /beneficial is probably recommended or indicated may/might be considered may/might be reasonable usefulness/effectiveness is unknown/unclear/uncertain or not well established : No enefit is not recommended is not indicated should not be performed/ administered/other is not useful/beneficial/ effective : Harm potentially harmful causes harm associated with excess morbidity/mortality should not be performed/ administered/other Estimate of ertainty (precision) of Treatment Effect meta-analyses meta-analyses LEVEL Recommendation that Limited populations evaluated a procedure or treatment is Data derived from a single randomized trial or Evidence from single nonrandomized studies randomized trial or nonrandomized studies LEVEL Recommendation that Very limited populations procedure or treatment is evaluated a Only consensus opinion of experts, case studies, or Only expert opinion, case standards of care studies, or standard of care trials or meta-analyses Recommendation in favor of treatment or procedure being Some conflicting evidence from single randomized trial or nonrandomized studies Recommendation in favor of treatment or procedure being Only diverging expert opinion, case studies, or standard of care Recommendation's usefulness/efficacy less well established Only diverging expert opinion, case studies, or standard of care Recommendation that procedure or treatment is not and may be harmful Only expert opinion, case studies, or standard of care Recommendation's usefulness/efficacy less well established Greater conflicting evidence from single randomized trial or nonrandomized studies Recommendation that procedure or treatment is not and may be harmful Evidence from single randomized trial or nonrandomized studies ack to Table of ontents Estimate of ertainty (Precision) of Treatment Effect LEVEL A Multiple populations evaluated a Data derived from multiple randomized clinical trials or Recommendation that procedure or treatment is Sufficient evidence from multiple randomized trials or Recommendation in favor of treatment or procedure being Some conflicting evidence from multiple randomized Recommendation's usefulness/efficacy less well established Greater conflicting evidence from multiple randomized trails or meta-analyses Recommendation that procedure or treatment is not and may be harmful Sufficient evidence from multiple randomized trials of meta-analyses : Harm Excess ost w/o enefit or Harmful Harmful to Patients LASS enefit >>> Risk Procedure/Treatment SHOULD be performed/ administered LASS a enefit >> Risk Additional studies with focused objectives needed T S REASONALE to perform procedure/ administer treatment LASS b enefit Risk Additional studies with broad objectives needed; additional registry data would be helpful Procedure/Treatment MAY E ONSDERED : No benefit Not Helpful No Proven enefit Procedure/ Test Treatment LASS No enefit or LASS Harm Size of Treatment Effect
ack to Table of ontents Abbreviations 2-D, 2-dimensional, AVAi, aortic valve area indexed to body surface area; AVR, aortic valve replacement; P, blood pressure; KD, chronic kidney disease; NS, central nervous system;, lass of Recommendation; VA, cardiovascular accident (stroke); DLO 2, carbon dioxide diffusing capacity of the lungs; DSE, dobutamine stress echocardiography; EG, electrocardiogram, FEV1, forced expiratory volume in 1 second; G, gastrointestinal; GDMT, guideline-directed medical therapy; HF, heart failure; NR, international normalized ratio; LOE, Level of Evidence; LV, left ventricular; LVEF, left ventricular ejection fraction; m/s, meters per second; NYHA, New York Heart Association; ΔP, pressure gradient; PHTN, pulmonary hypertension, PROM, predicted risk of mortality; RT, randomized controlled trial; RV, right ventricular; STS, Society of Thoracic Surgeons; TAVR, transcatheter aortic valve replacement; TTE, transthoracic echocardiography/echocardiogram; VKA, vitamin K antagonist; V max, maximal velocity; y, year This resource is provided compliments of Edwards Lifesciences LL. t is intended for reference use only and healthcare providers must make clinical judgments based on their independent assessment of each patient. Edwards Lifesciences LL was not involved in the development of the underlying guidelines on which this publication is based. AR12009 Source Nishimura RA, Otto M, onow RO, arabello A, Erwin JP, Guyton RA, O Gara PT, Ruiz E, Skubas NJ, Sorajja P, Sundt TM, Thomas JD. 2014 AHA/A guideline for the management of patients with valvular heart disease: a report of the American ollege of ardiology/american Heart Association Task Force on Practice Guidelines. J Am oll ardiol 2014;63:e57 185. opublished in irculation 2014;129:e521-e643. Disclaimer This Guideline attempts to define principles of practice that should produce high-quality patient care. t focuses on the needs of primary care practice, but also is applicable to providers at all levels. This Guideline should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician after consideration of each individual patient situation. Neither G, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool. AVHDAS14081c 106 ommerce Street, Suite 105 Lake Mary, FL 32746 TEL: 407.878.7606 FAX: 407.878.7611 For additional copies: info@guidelineentral.com opyright 2014 All rights reserved