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Introduction and general comments 

Introduction and general comments
Chapter:
Introduction and general comments
Author(s):

Bernard Iung

, and Pieter Kappetein

DOI:
10.1093/med/9780198784906.003.0764
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date: 23 March 2019

This chapter provides the background information and detailed discussion of the data for the following current ESC Guidelines on: Introduction and general comments Management of Valvular Heart Disease - https://academic.oup.com/eurheartj/article/38/36/2739/4965046#115331287

This section was reviewed and edited by The Task Force for the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS) Authors/Task Force Members: Helmut Baumgartner (ESC Chairperson) (Germany), Volkmar Falk (EACTS Chairperson) (Germany), Jeroen J. Bax (The Netherlands), Michele De Bonis (Italy), Christian Hamm (Germany), Per Johan Holm (Sweden), Bernard Iung (France), Patrizio Lancellotti (Belgium), Emmanuel Lansac (France), Daniel Rodriguez Muñoz (Spain), Raphael Rosenhek (Austria), Johan Sjögren (Sweden), Pilar Tornos Mas (Spain), Alec Vahanian (France), Thomas Walther (Germany), Olaf Wendler (UK), Stephan Windecker (Switzerland), Jose Luis Zamorano (Spain)

Summary

Valvular heart disease (VHD) is frequent is industrialized countries and its prevalence increases with age due to the predominance of degenerative aetiology. Clinical approach is paramount for evaluation of the patient’s history, symptoms and for the detection of VHD by auscultation. Echocardiography plays a major role in diagnosis and assessment of severity and prognosis. Other investigations are mainly non-invasive and include stress testing, multimodality imaging and biomarkers. Risk stratification is essential to weigh the risk of intervention against the expected natural history of VHD. It should include risk scores, keeping in mind their limitations, in particular in the elderly. Heart valve centres are required to deliver high-quality care and provide adequate training. Non-vitamin K antagonist oral anticoagulants may be used in patients with atrial fibrillation and aortic stenosis, aortic regurgitation, mitral regurgitation, or aortic bioprostheses beyond 3 months after implantation, but are contraindicated in mitral stenosis and mechanical valves.

Valvular heart disease (VHD) accounts for a significant burden in the community and predominates in elderly patients (see Chapter 56.7), thereby raising particular problems for the evaluation of the risk:benefit ratio of interventions. Interventions for VHD are the only effective therapy for improving survival. Valvular interventions have been reoriented with the development of less invasive approaches, in particular transcatheter interventions.

This chapter will provide an updated review of the main aspects of each acquired valve disease in adults and include patients who have previously undergone valve surgery. It will also present principles of management with regards to diagnosis and treatment that are derived from the most recent guidelines.

Epidemiology

The age-adjusted prevalence of moderate or severe VHD has been estimated at 2.5% (95% confidence interval 2.2–2.7%) in a population-based series on 11,911 patients comprising systematic echocardiographic examination.1 This prevalence was highly dependent on age and increased markedly after the age of 65 to reach 13% after 75. Age distribution of VHD in industrialized countries is related to the sharp decrease in the incidence of acute rheumatic fever and, therefore, of rheumatic heart disease.2 This has been compensated for by an important increase in the prevalence of so-called degenerative VHD, a term encompassing heterogeneous pathophysiology and lesions but sharing an increased prevalence with age. Degenerative VHDs are mainly calcific aortic disease causing aortic stenosis (AS). They frequently involve the mitral annulus but most often without significant haemodynamic consequences. Degenerative lesions of the mitral valve and the aortic valve and root are the most frequent cause of primary mitral regurgitation (MR) and aortic regurgitation (AR).3 Other aetiologies are infective endocarditis (IE), inflammatory, drug-induced, radiation-induced, and congenital VHD.

Calcific aortic disease occurs on normal or, more frequently, on bicuspid aortic valves. The early stage is aortic sclerosis, which progresses slowly to significant AS. Epidemiological studies reported consistent estimations of the prevalence of significant AS (Figure 35.1.1).1,4,5,6,7,8 The annual incidence of AS is estimated around 5 per 1000.6 Due to population ageing and the absence of prevention, the number of elderly patients with AS is expected to be multiplied by two to three within the next 50 years.9,10

Figure 35.1.1 Prevalence of aortic stenosis according to age in population-based series from the United States or Europe: Lindroos et al. (Finland),4 Stewart et al. (USA),5 Nkomo et al. (USA),1 Eveborn et al. (Norway),6 and Danielsen et al. (Iceland)7.

Figure 35.1.1 Prevalence of aortic stenosis according to age in population-based series from the United States or Europe: Lindroos et al. (Finland),4 Stewart et al. (USA),5 Nkomo et al. (USA),1 Eveborn et al. (Norway),6 and Danielsen et al. (Iceland)7.

Lindman BR, Clavel MA, Mathieu P, Iung B, Lancellotti P, Otto CM, Pibarot P. Calcific aortic stenosis. Nat Rev Dis Primers 2016;2:16006.

Mitral valve prolapse is the most frequent cause of primary MR. Its prevalence is estimated at 2.4% but less than 5% of cases are associated with severe MR.11 There are presently no reliable estimations of the prevalence of secondary MR, although it is likely to account for a high number of cases in the general population.12

The prevalence of moderate or severe AR is estimated at less than 1%.2,12 Degenerative AR may be due to abnormalities of the aortic valve or ascending aortic aneurysm, or both, on a tricuspid or bicuspid aortic valve.

Mitral stenosis (MS) is the only VHD which remains mainly of rheumatic origin, which explains its decline in industrialized countries, with a prevalence estimated at 0.1%.1

The annual incidence of IE is estimated between 15 and 80 cases per million from population-based studies in industrialized countries.12 Over the last decades, IE has been characterized by an increase in patient age and in the percentage of cases due to staphylococci, which is now the most frequent responsible microorganism.13,14

Rheumatic fever remains endemic in developing countries, where rheumatic heart disease is highly prevalent, with most estimations ranging between 5 and 10 cases per 1000 subjects according to clinical screening in school-aged children.15 Prevalence rates are approximately tenfold higher when assessed using systematic echocardiographic screening.16 The prevalence of rheumatic heart disease is largely associated with socioeconomic status. Consequently, the distribution between rheumatic and degenerative VHD follows an intermediate pattern in emerging countries, as illustrated by a Turkish survey in which 46% of cases of VHD were of rheumatic origin and 29% of degenerative origin.17 Rheumatic heart disease remains, however, present in industrialized countries due to migrations.3

In the Euro Heart Survey, patients who had undergone previous valvular intervention accounted for as many as 28% of patients referred to hospital for VHD.3 The percentage of valvular surgery has gradually increased in the decade 2000–2010 at the expense of coronary artery bypass grafting,18 and this was associated with older age and increased frequency of co-morbidities.19 Besides surgery, the number of transcatheter interventions is progressively increasing, mainly in AS, and is likely to continue to increase in the near future.

General principles of patient management

Patient evaluation

The aims of the evaluation of patients with VHD are to diagnose, quantify, and assess the mechanism of VHD as well as its consequences. The consistency between the results of diagnostic investigations and clinical findings should be checked at each step in the decision-making process (see Chapter 58.3). Decision-making should be made by a heart team with a particular expertise in VHD, including cardiologists, cardiac surgeons, imaging specialists, anaesthesiologists, and, if needed, general practitioners, geriatricians, and heart failure (HF), electrophysiology, or intensive care specialists. This heart team approach is particularly advisable in the management of high-risk patients and is also important for other subsets such as asymptomatic patients where the evaluation of valve reparability is a key component in decision-making.

Decision-making can be summarized according to the approach described in Box 35.1.1.

* Life expectancy should be estimated according to age, gender, co-morbidities, and country-specific life expectancy.

Finally, indications for intervention and which type of intervention should be chosen rely mainly on the comparative assessment of spontaneous prognosis and the results of intervention according to the characteristics of VHD and co-morbidities.

Clinical evaluation

The aim of obtaining a case history is to assess symptoms and to evaluate for associated co-morbidity. The patient is questioned on his/her lifestyle to detect progressive changes in daily activity in order to limit the subjectivity of symptom analysis, particularly in the elderly (see also Section 1). In chronic conditions, adaptation to symptoms occurs. Repeated clinical evaluations are useful in this setting. Symptom development is often a driving indication for intervention. Patients who currently deny symptoms, but have been treated for HF, should be classified as symptomatic after exclusion of other potential causes of HF unrelated to valve disease. The reason for functional limitation and its degree, together with its relation to the underlying valvular problem, should be documented in the records. In the presence of cardiac and extracardiac co-morbidities it is important to elucidate the true cause of the symptoms. In patients receiving chronic anticoagulant therapy, it is necessary to assess the compliance with treatment and look for evidence of thromboembolism or bleeding. It is also necessary to search for minor complications, such as transient ischaemic attack or minor bleeding, which are frequently overlooked by the patient.

Clinical examination, in particular auscultation, plays a major role in the detection of VHD in asymptomatic patients. It is the first step in the definitive diagnosis of VHD and the assessment of its severity, keeping in mind that a low-intensity murmur may coexist with severe VHD, particularly in the presence of HF. In patients with heart valve prostheses, it is necessary to be aware of any change in murmur or prosthetic valve sounds.20 Clinical signs of HF are usually encountered at advanced stages of VHD.21 Clinical examination also contributes to the search for co-morbidities.

Electrocardiogram and chest X-ray complete clinical evaluation. Analysis of pulmonary vascular distribution is useful in the interpretation of dyspnoea.

Echocardiography

Echocardiography is the key technique used to confirm the diagnosis of VHD as well as to assess its severity and prognosis (see Chapter 10.13). It should be performed and interpreted by properly trained personnel.22 It is indicated in every patient with a murmur, unless no suspicion of valve disease is raised after the clinical evaluation.

The evaluation of the severity of stenotic VHD should combine the assessment of valve area with flow-dependent indices such as mean pressure gradient and maximal flow velocity.23 Flow-dependent indices add further information and have a prognostic value.

The assessment of valvular regurgitation should combine different indices including quantitative measurements, such as the vena contracta and effective regurgitant orifice area (EROA), which are less dependent on flow conditions than colour Doppler jet size (Table 35.1.1).24 However, all quantitative evaluations have limitations. In particular, they combine a number of measurements, are highly sensitive to errors of measurement, and are highly operator dependent; therefore, their use requires experience and integration of a number of measurements rather than reliance on a single parameter. It is necessary to be aware of potential errors of measurements. Detailed comments for specific parameters are provided in the chapters in the rest of Section 35.

Table 35.1.1 Echocardiographic criteria for the definition of severe valve regurgitation: an integrative approach

Aortic regurgitation

Mitral regurgitation

Tricuspid regurgitation

Qualitative

Valve morphology

Abnormal/flail/large coaptation defect

Flail leaflet/ruptured papillary muscle/large coaptation defect

Abnormal/flail/large coaptation defect

Colour flow regurgitant jet

Large in central jets, variable in eccentric jets*

Very large central jet or eccentric jet adhering, swirling, and reaching the posterior wall of the left atrium

Very large central jet or eccentric wall impinging jet*

CW signal of regurgitant jet

Dense

Dense/triangular

Dense/triangular with early peaking (peak <2 m/s in massive TR)

Other

Holodiastolic flow reversal in descending aorta (EDV >20 cm/s)

Large flow convergence zone*

Semiquantitative

Vena contracta width (mm)

>6

≥7 (>8 for biplane)

≥7*

Upstream vein flow§

Systolic pulmonary vein flow reversal

Systolic hepatic vein flow reversal

Inflow

E-wave dominant ≥1.5 m/s

E-wave dominant ≥1 m/s**

Other

Pressure half-time <200 ms

TVI mitral/TVI aortic >1.4

PISA radius >9 mm ††

Quantitative

Primary

Secondary‡‡

EROA (mm2)

≥30

≥40

≥20

≥40

R vol (mL/beat)

≥60

≥60

≥30

≥45

+ enlargement of cardiac chambers/vessels

LV

LV, LA

RV, RA, inferior vena cava

* At a Nyquist limit of 50–60 cm/s.

Pressure half-time is shortened with increasing left ventricular diastolic pressure, vasodilator therapy, and in patients with a dilated compliant aorta, or lengthened in chronic aortic regurgitation.

For average between apical four- and two-chamber views.

§ Unless other reasons for systolic blunting (atrial fibrillation, elevated atrial pressure).

In the absence of other causes of elevated left atrial pressure and of mitral stenosis.

** In the absence of other causes of elevated right atrial pressure.

†† Baseline Nyquist limit shift of 28 cm/s.

Ȃ‡ Different thresholds are used in secondary MR where an EROA >20 mm2 and regurgitant volume >30 mL identify a subset of patients at increased risk of cardiac events.

CW, continuous wave; EDV, end-diastolic velocity; EROA, effective regurgitant orifice area; LA, left atrium; LV, left ventricle; PISA, proximal isovelocity surface area; RA, right atrium; RV, right ventricle; R vol, regurgitant volume; TR, tricuspid regurgitation; TVI, time–velocity integral.

Adapted from Lancellotti P, Tribouilloy C, Hagendorff A, Popescu BA, Edvardsen T, Pierard LA, Badano L, Zamorano JL. Recommendations for the echocardiographic assessment of native valvular regurgitation: an executive summary from the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 2013;14:611–44.

Thus, when assessing the severity of VHD, it is necessary to check consistency between the different echocardiographic measurements as well as the anatomy and mechanisms of VHD. It is also necessary to check their consistency with the clinical assessment.

Echocardiography should include a comprehensive evaluation of all valves, looking for associated valve diseases and the aorta.

Indices of left ventricular (LV) enlargement and function are strong prognostic factors and play an important role in decision-making for interventions in regurgitant VHD. While diameters allow a less complete assessment of LV size than volumes, their prognostic value has been studied more extensively. LV dimensions should be indexed to body surface area. The use of indexed values is of particular interest in patients with a small body size, but should be avoided in patients with severe obesity (body mass index >40 kg/m2). Indices derived from Doppler tissue imaging and strain assessments seem to be of potential interest for the detection of early impairment of LV function, but lack validation of their prognostic value for clinical endpoints.25,26

Finally, the pulmonary pressures should be evaluated as well as right ventricular (RV) function.27 There are several simple and reproducible methods of assessing RV systolic function such as fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), and pulsed tissue Doppler S′. Combining more than one measure of RV function, such as S′ and RV index of myocardial performance may more reliably distinguish normal from abnormal function.

Three-dimensional echocardiography is useful for assessing anatomical features which may have an impact on the type of intervention chosen, particularly on the mitral valve (see also Chapter 10.6).28,29

Transoesophageal echocardiography (TOE) should be considered when transthoracic echocardiography (TTE) is of suboptimal quality or when thrombosis, prosthetic dysfunction, or endocarditis is suspected (see Chapter 10.9). Intraprocedural TOE is used to monitor the results of surgical valve repair or percutaneous procedures. High-quality intraoperative TOE is mandatory for all valve operations to document normal function of the implanted prosthesis, document the absence of paravalvular leaks, and assess the result of a repair procedure. Three-dimensional TOE offers a more detailed examination of valve anatomy than two-dimensional echocardiography and is useful for the assessment of complex valve problems as well as for the determination of feasibility of percutaneous intervention.

Other non-invasive investigations

Stress testing

Stress testing is considered here for the evaluation of VHD or its consequences (or both), but not for the diagnosis of associated coronary artery disease (CAD). Predictive values of functional tests used for the diagnosis of CAD may not apply in the presence of VHD and are generally not used in this setting.30 In addition, exercise testing is contraindicated in symptomatic AS.

  • Exercise ECG: the primary purpose of exercise testing is to unmask the objective occurrence of symptoms in patients who claim to be asymptomatic or have doubtful symptoms. Exercise testing also has an additional value for risk stratification in AS.31 Exercise testing will also determine the level of authorized physical activity, including participation in sports.

  • Exercise echocardiography (see also Chapter 10.4): exercise echocardiography may provide additional information in order to better identify the cardiac origin of dyspnoea, which is a rather unspecific symptom, by showing, for example, an increase in the degree of mitral regurgitation/aortic gradient and in systolic pulmonary pressures.32 It has a diagnostic value in transient ischaemic MR which may be overlooked in investigations at rest. The prognostic impact of exercise echocardiography has been mainly shown for AS and MR.33

  • Other stress tests: the search for flow reserve (also called contractile reserve) using low-dose dobutamine stress echocardiography is useful for assessing severity and operative risk stratification in AS with impaired LV function and low gradient as well as to assess the potential of reverse remodelling in patients with HF and functional MR after a mitral valve procedure.34

Cardiac magnetic resonance

In patients with inadequate echocardiographic quality or discrepant results, cardiovascular magnetic resonance (CMR) should be used to assess the severity of valvular lesions, particularly regurgitant lesions, and to assess ventricular volumes, systolic function, abnormalities of the ascending aorta, and myocardial fibrosis, as CMR assesses these parameters with higher reproducibility than echocardiography.35 CMR is the reference method for the evaluation of RV volumes and function and is therefore useful to evaluate the consequences of tricuspid regurgitation (TR).36

Computed tomography

Multislice computed tomography (MSCT) may contribute to the evaluation of the severity of valve disease, particularly in AS, either indirectly by quantifying valvular calcification37,38 or directly through the measurement of valve planimetry.39 CT is the most accurate technique to assess the extension, severity, and location of valvular calcification, providing essential information for pre-procedural planning. It is widely used to assess the dimensions and location of aneurysms of the ascending aorta and aortic arch.40 Due to its high negative predictive value, MSCT may be useful to exclude CAD in patients who are at low risk of atherosclerosis.39,41

MSCT plays an important role in the work-up of high-risk patients with AS considered for transcatheter aortic valve implantation (TAVI), and provides valuable information for pre-procedural planning before intervention.42,43 New applications of MSCT such as dual-energy CT and spectral CT may be of additional value for evaluation of cardiac function in VHD in the future, but at present the data is limited.44 The risk of radiation exposure, and of renal failure due to contrast injection, should, however, be taken into consideration.

Both CMR and MSCT require the involvement of radiologists/cardiologists with special expertise in VHD imaging.45

Cinefluoroscopy

Cinefluoroscopy is more specific than echocardiography for assessing valvular or annular calcification. It is also useful for assessing the kinetics of the leaflets of a mechanical prosthesis.46

Biomarkers

B-type natriuretic peptide serum levels have been shown to be related to functional class and prognosis, particularly in AS and MR.47 Natriuretic peptides may also be of additional value in risk stratification, particularly in asymptomatic patients.48

Invasive investigations

  • Coronary angiography: coronary angiography is indicated for the detection of associated CAD when surgery is planned and determines if concomitant coronary revascularization is indicated (Table 35.1.2).30 However, MSCT has become a valuable non-invasive diagnostic tool in patients who are at low risk or intermediate risk of atherosclerosis.30

  • Coronary angiography can be omitted in young patients with no atherosclerotic risk factors (men <40 years and premenopausal women) and in rare circumstances when its risk outweighs benefit (e.g. in acute aortic dissection, a large aortic vegetation in front of the coronary ostia, or occlusive prosthetic thrombosis in an unstable haemodynamic condition).

  • Cardiac catheterization: the measurement of pressures and cardiac output or the assessment of ventricular performance and valvular regurgitation by ventricular angiography or aortography are restricted to the rare situations where non-invasive evaluation is inconclusive or discordant with clinical findings. Given its potential risks, cardiac catheterization to assess haemodynamics should not be performed routinely with coronary angiography. When elevated pulmonary pressure is the only criterion to support the indication for surgery, confirmation of echo data by invasive measurement is recommended.

Table 35.1.2 Management of coronary artery disease in patients with valvular heart disease

Introduction and general comments

a Class of recommendation. b Level of evidence.

* Multislice computed tomography may be used to exclude coronary artery disease in patients who are at low risk of atherosclerosis.

† Chest pain, abnormal non-invasive testing.

‡ ≥50% can be considered for left main stenosis.

CABG, coronary artery bypass grafting; PCI, percutaneous coronary intervention; TAVI, transcatheter aortic valve implantation; VHD, valvular heart disease.

Adapted from Windecker S, Kolh P, Alfonso F, et al. 2014 ESC/EACTS Guidelines on myocardial revascularization: The Task Force on Myocardial Revascularization of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Developed with the special contribution of the European Association of Percutaneous Cardiovascular Interventions (EAPCI). Eur Heart J 2014;35:2541–619.

Assessment of co-morbidity

The choice of specific examinations to assess co-morbidity is directed by the clinical evaluation. The most frequently encountered co-morbidities are peripheral atherosclerosis, renal and hepatic dysfunction, and chronic obstructive pulmonary disease. Specific validated scores enable the assessment of cognitive and functional capacities which have important prognostic implications in the elderly. The expertise of geriatricians is particularly helpful in this setting.

Risk stratification

The use of risk stratification scores is a useful tool in helping cardiologists and cardiac surgeons take decisions regarding valvular interventions, especially in patients at increased risk of perioperative morbidity and mortality.49 The decision to intervene in a patient with VHD relies on an individual risk:benefit analysis, suggesting that improvement of prognosis as compared with natural history outweighs the risk of intervention (Table 35.1.3)50,51,52,53 and its potential late consequences, particularly prosthesis-related complications.

Table 35.1.3 Operative mortality after surgery for valvular heart disease in all comers

STS (2014)50

UK (2012)51

Germany (2014)52,53

Aortic valve replacement, no CABG (%)

  • 2.4

  • (29,158)

  • 1.7

  • (4561)

  • 2.7

  • (11,881)

Aortic valve replacement + CABG (%)

  • 3.9

  • (18,016)

  • 4.0

  • (3263)

  • 4.5

  • (3462)

Mitral valve repair, no CABG (%)

  • 1.2

  • (8658)

  • 2.0

  • (1456)

  • 1.6

  • (3621)

Mitral valve repair + CABG (%)

  • 5.1

  • (4205)

  • 5.6

  • (588)

  • 6.6

  • (1854)

Mitral valve replacement no CABG (%)

  • 4.9

  • (6857)

  • 4.2

  • (638)

  • 8.4

  • (2001)

Mitral valve replacement +CABG (%)

  • 9.9

  • (2582)

  • 11.6

  • (232)

  • 16.4

  • (786)

Reoperations are excluded in the STS and UK reports but not in the data from Germany.

() = number of patients.

CABG, coronary artery bypass grafting; STS, Society of Thoracic Surgeons (USA). Mortality for STS includes first and redo interventions50; UK, United Kingdom.

Data from references.50,51,52,53

Operative mortality can be estimated by various multivariable scoring systems using combinations of risk factors.54 The formerly used EuroSCORE55 (European System for Cardiac Operative Risk Evaluation, http://www.euroscore.org/calc.html) has been shown to consistently overestimate operative mortality and its calibration of risk is poor.56 Consequently, it should no longer be used to guide decision-making. The EuroSCORE II57 and the STS58,59 (Society of Thoracic Surgeons) score (http://209.220.160.181/STSWebRiskCalc261/) have been shown to more accurately discriminate high- and low-risk patients as well as better calibration to predict individual postoperative outcome and they achieve comparable performance in valvular surgery.60,61,62,63,64,65 The latter has the advantage of being specific to VHD. However, the calibration of the EuroSCORE II is less satisfying in high-risk patients.66 Additionally, these scores have shown variable results in predicting the outcomes of intervention in TAVI.67 New scores have been developed to estimate the risk of 30-day mortality in patients undergoing TAVI, with better accuracy and discrimination.68,69 It remains, however, essential not to rely on a single number to assess patient risk, nor to determine unconditionally the indication and type of intervention. The predictive performance of risk scores may be improved by repeated recalibration of scores over time, as is the case for STS and EuroSCORE with the EuroSCORE II, by the addition of variables, in particular indices aimed at assessing functional and cognitive capacities and frailty in the elderly,70 or by the design of separate risk scores for particular subgroups, such as the elderly or patients undergoing combined valvular and coronary surgery.

The natural history of VHD should ideally be derived from contemporary series, but no scoring system is available in this setting. Certain validated scoring systems enable a patient’s life expectancy to be estimated according to age, co-morbidities, and indices of cognitive and functional capacity.71 Expected quality of life should also be considered. The futility of interventions in patients unlikely to benefit from the treatment has to be taken into consideration, particularly for TAVI and mitral edge-to-edge repair.

Local resources should also be taken into account, in particular the availability of valve repair, as well as outcomes after surgery and percutaneous intervention in the specified centre.72 Depending on local expertise, patient transfer to a more specialized centre should be considered for procedures such as complex valve repair.73

Finally, a decision should be reached through the process of shared decision-making, first by a multidisciplinary heart team discussion, then by informing the patient thoroughly, and finally by deciding with the patient and family which treatment option is optimal.74

Special considerations in elderly patients

Older age and frequent co-morbidities increase the risk of interventions and have a negative impact on life expectancy, thereby making risk:benefit analysis of interventions more difficult than in younger patients (see Chapters 56.7 and 56.11). This is of particular importance for the choice between surgery, TAVI, and medical therapy in AS, which is the most prevalent VHD in the elderly. Chronic lung disease, renal insufficiency, liver disease, and vascular disease are the most frequent organ co-morbidities which have a negative impact on early and late results of surgery or TAVI57,58,59,68,69,75 and also impair life expectancy regardless of heart disease.71,76

Chronic lung disease impairs immediate and late survival after valvular surgery and TAVI (see Chapter 23.3).77,78,79,80 Poor mobility, as assessed by the 6-minute walk test, and oxygen dependency are the main factors associated with increased mortality after TAVI.79,80 Spirometric variables are associated with pulmonary complications but should be interpreted with other factors, in particular reflecting functional impairment.

There is a gradual relationship between the impairment of renal function and increased mortality after valvular surgery, TAVI, and transcatheter mitral edge-to-edge repair.81,82,83,84 This relationship is particularly marked when glomerular filtration rate is less than 30 mL/min.83

Hepatic insufficiency is a rare condition in surgical databases and its impact is therefore difficult to assess.49 Limited retrospective data have shown an association between the Model for End-stage Liver Disease (MELD) score and morbi-mortality after cardiac surgery.85

Coronary, cerebrovascular, and lower limb artery diseases have a negative impact on early and late survival after surgery and TAVI.82,86

Besides specific organ co-morbidities, there is growing interest in the assessment of frailty, which corresponds to a syndrome of decreased reserve and resistance to stressors and is an overall marker of impairment of functional, cognitive, and nutritional status (see Chapter 56.4).87 Frailty is associated with increased morbi-mortality after surgery and TAVI.75,88,89,90,91 This association is stronger in elderly patients undergoing TAVI than in younger patients undergoing cardiac surgery.88 Frailty also predicts functional decline after TAVI.92 The assessment of frailty should not rely on a subjective approach such as the ‘eye ball test’ but rather on the combination of different objective estimates. A number of tools are available for assessing frailty.93 However, it is presently not possible to recommend a standardized and simple method for assessing frailty, in particular through the use of a limited subset of geriatric scales which could be selected according to their own prognostic value.

Multivariate risk scores are the only way to combine the respective prognostic weights of co-morbidities. However, the predictive performance of risk scores is decreased in elderly or high-risk patients.49,66,94,95 There is limited experience concerning the addition of variables reflecting frailty to co-morbidities in risk scores. The interpretation is difficult due to the absence of a standardized assessment. In addition, available predictive analyses of early or short-term morbi-mortality showed a modest improvement of discrimination when adding frailty estimates.96,97

In current practice, the search for co-morbidities is oriented by clinical evaluation. Respiratory, renal, hepatic, and vascular co-morbidities should be systematically searched for and quantified in the elderly. The involvement of organ specialists is particularly needed to determine if a single co-morbidity contraindicates an intervention. The assessment of frailty by a geriatrician is particularly recommended when it has an important impact in decision-making. However, the most frequent situation is the conjunction of different co-morbidities, which individually do not firmly contraindicate intervention. In these cases, the only means to assess the overall impact of co-morbidities is the use of risk scores. The limitations of the predictive performance of risk scores in high-risk patients should be kept in mind and highlight the importance of a multidisciplinary assessment by the heart team, weighing the risk of intervention against the natural history of the VHD. It is particularly important in the elderly that the patient and relatives are involved through a shared decision-making process.74

Endocarditis prophylaxis

Patients with a prosthetic valve, including transcatheter valves, or in whom valve repair has been performed using prosthetic material, and those with previous IE are at higher risk of IE and present higher morbidity and mortality from IE (see also Chapter 7.8).98 Hence, current European Society of Cardiology guidelines indicate that antibiotic prophylaxis should be considered for high-risk procedures in these patients.99 Recommendations regarding dental and cutaneous hygiene and strict aseptic measures during any invasive procedure are advised in this population. Additionally, antibiotic prophylaxis should be considered in dental procedures involving manipulation of the gingival or periapical region of the teeth or manipulation of the oral mucosa.99

Prophylaxis for rheumatic fever

Prevention of rheumatic heart disease should preferably be oriented to preventing the first attack of acute rheumatic fever. Improvements in hygiene, living conditions, and access to medical care significantly impact its incidence (see also Chapters 7.6 and 26.2). Additionally, treatment of group A streptococci sore throat by oral or injectable penicillin is key in primary prevention.15 In patients with rheumatic heart disease, secondary long-term prophylaxis against rheumatic fever is recommended to prevent recurrent episodes and consequent progression of the disease (see Chapter 26.6). Three- to four-weekly injections of intramuscular benzathine penicillin, preferred to oral regimens due to higher efficacy in prevention of relapse, is recommended for at least 10 years after the last episode of acute rheumatic fever or until 40 years of age, whichever is the longest. Lifelong prophylaxis should be considered in high-risk patients according to the severity of VHD and exposure to group A streptococci.100,101

Concept of valve clinic, heart team, and centres of excellence

When patients with VHD are referred in a timely manner, an intervention carries a lower risk and is usually more successful in improving survival and reducing symptoms. The main advantage of a specialist clinic is to deliver better quality of care than in a general clinic as a result of greater volumes associated with specialization of training, continuing education, and clinical interest. In specialized clinics, guidelines are more consistently applied and the number of inappropriate examinations is reduced. Specialization will also result in timely referral of patients before irreversible adverse effects occur and techniques with a steep learning curve are more likely to be applied in hospital with more experience (e.g. mitral valve repair) (see also Chapter 58.3).

The mechanisms of how valve clinics can optimize care are multiple: adequate evaluation of the patient, monitoring of the disease at appropriate time intervals, determining the right time and type for valve intervention, referring to the right surgeon or interventional cardiologist, and assessing the results after the intervention.102

A centre of excellence should provide a multidisciplinary team (heart team) that meets on a regular basis, works with standard operating procedures, and implements current guidelines. Risk assessment should be performed by application of risk scores such as the STS score or EuroSCORE II and taking other conditions not captured by these risk scores into account. The collaborative approach between cardiologists, surgeons, specialists in imaging, and anaesthesiologists should also include the judgement of other specialist such as intensivists or geriatricians as required. Expert imaging including echocardiography, computed tomography, and magnetic resonance imaging is essential and exercise tests should be readily available if needed to assess valvular lesions under exercise conditions, assess potential for reverse remodelling, and allow for precise pre-procedural planning of surgery or interventions.103 Valve intervention should only be carried out in those hospitals where there is both a cardiology and a cardiac surgery department on site.

There is no formal European qualification process to establish competency in VHD and there is controversy with regard to adequate hospital volumes and individual surgical or interventional case load. For most valve interventions, there is both an effect of the number of cases performed per year and per surgeon or interventional cardiologist as well as per hospital.104,105,106,107,108 For both aortic and mitral valve replacement, a trend over time for adjusted odds ratios of mortality in very low-volume hospitals to very high-volume hospitals from 2000 to 2008 favouring high-volume hospitals has been observed in the United States.109 The precise numbers of procedures per individual surgeon/interventionalist or hospital required to provide high-quality care, however, remain controversial and more scientific data are required before solid recommendations can be provided. Experience in the full spectrum of surgical procedures including valve replacement; aortic root surgery; mitral, tricuspid, and aortic valve repair; repair of complicated valve endocarditis such as root abscess; and treatment of atrial fibrillation (AF), as well as surgical myocardial revascularization must be available. The spectrum of interventional procedures in addition to TAVI should include mitral valvuloplasty, mitral valve repair (edge-to-edge), closure of atrial septal defects, closure of paravalvular leaks, and left atrial appendage closure, as well as percutaneous coronary intervention. Expertise in interventional and surgical management of vascular diseases and complications must be available.

A Heart Valve Centre (Box 35.1.2) should encounter structured training programmes for physicians and staff. Standard operating procedures in line with current guidelines should be implemented and updated on a regular basis. Team members should be involved with research and teaching and membership of a specialized society. A database to monitor outcomes which is available for regular internal and external audit should be available. Participation in national and European registries should be mandatory.

Adapted from Lancellotti P, Rosenhek R, Pibarot P, Iung B, Otto CM, Tornos P, Donal E, Prendergast B, Magne J, La Canna G, Pierard LA, Maurer G. ESC Working Group on Valvular Heart Disease position paper – heart valve clinics: organization, structure, and experiences. Eur Heart J 2013;34:1597–606.

Management of associated conditions

Coronary artery disease

The use of stress tests to detect CAD associated with severe VHD is discouraged because of their low diagnostic value and potential risks.

A summary of the management of associated CAD is given in Table 35.1.2 and detailed in specific guidelines.30 Significant coronary disease generally leads to combined coronary artery bypass grafting when valvular surgery is indicated. Hybrid approaches combining percutaneous coronary intervention and valvular surgery have been proposed but experience remains limited and the management for antithrombotic therapy is difficult in this setting.

Atrial fibrillation and anticoagulation

Oral anticoagulation with a target international normalized ratio of 2–3 is recommended in patients with native VHD and any type of AF, taking the bleeding risk into account (see also Section 6 and Chapter 20.7).110 A higher level of anticoagulation may be necessary in specific patients with valve prostheses (see section on Prosthetic Valves).

Non-vitamin K antagonist oral anticoagulants (NOACs) are approved only for non-valvular AF, but there is no uniform definition of this term (Chapter 6.11).111 Recent subgroups analyses of randomized trials on AF support the use of rivaroxaban, apixaban, dabigatran, and edoxaban in patients with AS, AR, or MR presenting with AF.112,113,114,115 The use of NOACs is discouraged in patients who have AF associated with moderate to severe MS given the lack of data and the high thromboembolic risk.116,117 Despite the absence of data, NOACs may be used in patients who have AF associated with a bioprosthesis after the third postoperative month.118 NOACs are strictly contraindicated in patients with mechanical prostheses (see Chapter 35.9 ‘Prosthetic valves’).119

Except in cases where AF causes haemodynamic compromise, cardioversion is not indicated before intervention in patients with severe VHD, as it does not restore a durable sinus rhythm. Cardioversion should be attempted soon after successful intervention, unless in long-standing chronic AF.

Surgical ablation of AF combined with mitral valve surgery is effective in reducing the incidence of AF, but at the expense of more frequent pacemaker implantation, and has no impact on short-term survival (see also Chapter 41.14).120 Surgical ablation should be considered in patients with symptomatic AF and may be considered in patients with asymptomatic AF if feasible with minimal risk. The decision should be individualized according to clinical variables, such as age, the duration of AF, and left atrial size.

For patients with AF and risk factors for stroke, long-term oral anticoagulation is therefore currently recommended although surgical ablation of AF and/or surgical left atrial appendage excision or exclusion may have been performed.110

Recommendations for the management of AF are summarized in Table 35.1.4.

Table 35.1.4 Management of atrial fibrillation in patients with valvular heart disease

Introduction and general comments

a Class of recommendation. b Level of evidence. c Reference(s) supporting class I (A + B) and IIa + IIb (A + B) recommendations.

AF, atrial fibrillation; NOAC, non-vitamin K antagonist oral anticoagulants; VKA, vitamin K antagonists.

Data from references110,111,119,121.

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Further Reading

Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP, Iung B, Otto CM, Pellikka PA, Quinones M.Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. Eur J Echocardiogr 2009;10:1–25.Find this resource:

Edwards FH, Cohen DJ, O’Brien SM, Peterson ED, Mack MJ, Shahian DM, Grover FL, Tuzcu EM, Thourani VH, Carroll J, Brennan JM, Brindis RG, Rumsfeld J, Holmes DR, Jr. Development and validation of a risk prediction model for in-hospital mortality after transcatheter aortic valve replacement. JAMA Cardiol 2016;1:46–52.Find this resource:

Lancellotti P, Tribouilloy C, Hagendorff A, Popescu BA, Edvardsen T, Pierard LA, Badano L, Zamorano JL. Recommendations for the echocardiographic assessment of native valvular regurgitation: an executive summary from the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 2013;14:611–44.Find this resource:

Rosenhek R, Iung B, Tornos P, Antunes MJ, Prendergast BD, Otto CM, Kappetein AP, Stepinska J, Kaden JJ, Naber CK, Acarturk E, Gohlke-Barwolf C. ESC Working Group on Valvular Heart Disease Position Paper: assessing the risk of interventions in patients with valvular heart disease. Eur Heart J 2012;33:822–8.Find this resource: