Section 1: Epidemiology and aetiology
Despite significant advances in the diagnosis and management of valvular heart disease (VHD), it continues to impose a significant morbidity and mortality burden on resources.
This is mainly due to an increase in ageing population and the associated degenerative valvular lesions, as well as other comorbidities such as IHD and heart failure. VHD is more prevalent in the elderly (13% among those over 75 years compared with only 0.7% among those aged 18-44 years).1 Prevalence among those aged over 65 in the UK has been estimated at about 1m.2 The 2001 European VHD Survey showed that aortic stenosis (AS) is the most prevalent (43%), followed by mitral regurgitation (MR, 32%), aortic regurgitation (AR, 13%) and mitral stenosis (MS, 12%).3 The prevalence of significant AS among the elderly is 1-3%, AR, <1%, and MR, 2%. Primary disorders of the other valves are rare among the elderly.4 This review focuses on the four most common disorders.
The epidemiology of VHD shows a male predominance of AS and MR and a female predominance of MS and rheumatic heart disease.3,4
Aetiology and classification
The aetiologies of individual valvular lesions are illustrated in table 1.
TABLE 1: AETIOLOGIES OF INDIVIDUAL VALVULAR LESIONS
|AS||Degenerative, bicuspid (congenital), rheumatic|
|AR||Degenerative, bicuspid (congenital), rheumatic
AR Rheumatic, infective endocarditis, aortic root disease
(aortic dissection, vasculitis, connective tissue
disorders such as Marfan's syndrome)
|MS||Mainly rheumatic, rare causes include malignant
carcinoid, rheumatoid arthritis, systemic lupus
erythematosus (SLE), Fabry disease
|MR||Mitral valve prolapse, rheumatic fever, infective
endocarditis, IHD, Marfan's syndrome, SLE
Section 2: Making the diagnosis
The clinical features vary depending on the valvular lesion (table 2).
It is crucial to correlate symptoms and clinical signs with results from investigations, as modern tests may uncover incidental and benign valvular lesions which may not have clinical relevance. Routine blood tests such as FBC may show anaemia due to lower GI blood loss in AS or due to infective endocarditis.
Raised inflammatory markers with fever and new murmur may indicate endocarditis. Chest X-ray may reveal cardiomegaly or pulmonary congestion.
ECG is crucial. AF can complicate most valvular lesions and worsens symptoms in AS and MS. Significant AS usually shows features of left ventricular hypertrophy on ECG. Echocardiography is crucial to establish the diagnosis and grade severity of the lesion.
MRI is useful if echocardiographic images are suboptimal.
TABLE 2: SIGNS AND SYMPTOMS OF INDIVIDUAL VHD
Symptoms (in moderate)
Signs (in moderate to severe VHD)
Angina, exertional syncope or dyspnoea
|Small and slow-rising pulse
Laterally displaced apical impulse
Aortic component of S2 reduced (degenerative form) and disappears in severe AS
Fourth heart sound (S4)
Ejection systolic murmur radiating to both carotids
|Exertional dyspnoea, paroxysmal nocturnal dyspnoea and orthopnoea||Collapsing pulse (water-hammer type), wide pulse pressure
Head-nodding with each heartbeat (de Musset sign)
‘Pistol-shot’ (systolic and diastolic) sounds over femoral artery
Apical impulse displaced laterally and inferiorly
Early diastolic decrescendo murmur
|Dyspnoea, cough, haemoptysis, chest pain, systemic embolism||Mitral facies – pink patches on the face
AF, JVP – prominent ‘a wave’ if sinus rhythm
Tapping apical impulse and diastolic thrill
Opening snap and low-pitched rumbling mid-diastolic murmur
|Dyspnoea||Brisk hyperdynamic pulse
Hyperdynamic cardiac impulse deviated laterally
Systolic click is due to mitral valve prolapse
Section 3: Managing the condition
Management strategies for individual valvular lesions are detailed in table 3. The indications for surgery depend on the valve and are beyond the scope of this article. Detailed evaluation of VHD should include assessment by a multidisciplinary team of cardiologists, cardiothoracic surgeons and intensivists to assess symptoms, severity of valve disorder and correlation between the two, as well as patient expectations, life expectancy, and the risk:benefit ratio of treatment.
Recent European Society of Cardiology guidelines recommend endocarditis prophylaxis only for high-risk patients (such as patients with prosthetic valve, previous endocarditis or congenital heart disease) undergoing high-risk procedures.
TABLE 3: MANAGEMENT OF INDIVIDUAL VALVULAR LESIONS
|Valvular lesion||Medical management||Surgical treatments|
|AS||Medical therapy does not improve outcome
Treatment of heart failure with diuretics, vasodilators
Modification of atherosclerotic risk factors
|Aortic valve replacement (AVR) recommended in patients with severe symptomatic AS, patients with severe AS undergoing CABG, surgery on ascending aorta or other valve or in asymptomatic patients with severe AS and left ventricular ejection fraction (LVEF) ≤50% or abnormal exercise test
Autograft of pulmonary valve and artery (Ross procedure) in young
Balloon valvuloplasty – as bridge to surgery or if patients require urgent major non-cardiac surgery
Percutaneous transcatheter aortic valve implant in patients unsuitable for conventional AVR
|AR||ACE inhibitors in patients with chronic severe AR and hypertension if surgery contraindicated
Beta-blockers delay aortic root dilation in Marfan’s syndrome
|Valve sparing aortic replacement in young patients (if combined aortic root dilation
Pulmonary autograft to replace aortic valve in young patients
Aortic valve replacement – used widely
|MS||Diuretics, nitrates, beta-blockers to improve symptoms
Anticoagulation if AF, embolism or left atrial thrombus
|Percutaneous/open mitral commissurotomy if suitable
Mitral valve replacement
|MR||Acute MR – vasodilators (nitroprusside, nitrate), diuretics, intra-aortic balloon pump
Chronic MR – role of medical therapy if HF
|Mitral valve repair when feasible
Mitral valve replacement
Section 4: Prognosis
Severe AS patients have a five-year survival risk of 15-50%.3 Predictors of poor prognosis include older age, associated atherosclerosis, echocardiographic features such as calcification and impaired LV function.
Acute severe AR has a poor prognosis in absence of intervention. Onset of symptoms in chronic severe AR also indicates 10-20% annual mortality without surgery.3 Among patients with normal LV function and without symptoms, LV end systolic diameter >5cm predicts a high risk of symptoms, heart failure or death.3
In MS onset of symptoms predicts poor prognosis without intervention. Pregnancy and AF worsen symptoms. Acute MR (usually due to chordal rupture) has a high mortality if not operated on. Factors predicting poor prognosis in chronic MR include increasing age and pulmonary hypertension.
Section 5: Case study
An 82-year-old man presented with a six-month history of reducing exercise tolerance due to shortness of breath, chest tightness and exertional syncope.
His background history included type 2 diabetes mellitus with nephropathy and neuropathy, COPD and hypertension.
Clinical examination revealed BP 150/72mmHg, heart rate 64bpm regular, grade 3 ejection systolic murmur radiating to the carotids and bilateral wheeze on chest auscultation.
ECG showed sinus rhythm with LV hypertrophy. Echocardiography showed a calcified aortic valve with a mean gradient of 66mmHg, severe left ventricular hypertrophy and LVEF of 40%. He was referred to the cardiologist and reviewed in the valve clinic by a multidisciplinary team. He was referred for pulmonary function tests, which showed FEV1/FVC ratio of 35%.
In view of his comorbidities, he was deemed unsuitable for conventional valve replacement and was placed on a waiting list for a percutaneous transcatheter aortic valve implant.
While awaiting the procedure, the patient developed acute pulmonary oedema requiring hospitalisation, IV diuretics, inotropic support and non-invasive ventilation. Following stabilisation, he had a transvalvular balloon valvuloplasty as an interim measure, reducing the transaortic mean gradient to 36mmHg.
Nine months after his initial presentation, he underwent a percutaneous transcatheter aortic valve implant via the trans-femoral route, complicated by a self-resolving femoral haematoma and a TIA.
He was discharged 25 days later. His follow-up a year post-procedure has shown persistent improvements in exercise tolerance, quality of life score and complete resolution of chest pain and syncope.
This case illustrates the role of percutaneous transcatheter aortic valve implant in management of patients with AS and severe comorbidities precluding conventional valve replacement. But percutaneous transcatheter aortic valve implant is a new technique that is not yet widely available and most patients will therefore have to wait before the procedure, during which decompensation may require stabilisation.
Section 6: Evidence base
Glower D, Ailawadi G, Argenziano M et al. EVEREST II randomized clinical trial: predictors of mitral valve replacement in de novo surgery or after the MitraClip procedure. J Thorac Cardiovasc Surg 2012; 143(4 Suppl): S60-3.
Leon MB, Smith CR, Mack M et al. Transcatheter aortic-valve implantation for aortic stenosis in patients who cannot undergo surgery. N Engl J Med 2010; 363: 1597-1607.
European Society of Cardiology 2012. Vahanian A, Alfieri O, Andreotti F et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J 2012; 33: 2451-96.
American College of Cardiology/American Heart Association. Bonow RO, Carabello BA, Chatterjee K et al. Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease. Circulation 2008; 118: e523-e661.
ESC. Summary of valvular heart disease. http://www.escardio.org/guidelines-surveys/ esc-guidelines/GuidelinesDocuments/GP_Card_VHD_2012.pdf
An excellent resource for GPs, offering concise advice on the management of patients with valvular heart disease.
ESC/EACTS. Guidelines on the management of valvular heart disease (version 2012).
CPD IMPACT: EARN MORE CREDITS
These further action points may allow you to earn more credits by increasing the time spent and the impact achieved.
Learn which factors are associated with a poor prognosis and consider which ones can be modified.
Perform a search on all of your patients with a diagnosis of mitral valve disease and audit how many have been referred for echocardiography or to a cardiologist.
Consider writing a new practice algorithm on the management of patients with heart valve disorders and present it at a practice meeting.
1. Nkomo VT, Gardin JM, Skelton TN et al. Burden of valvular heart diseases: a population-based study. Lancet 2006; 368: 1005-11.
3. Vahanian A, Alfieri O, Andreotti F et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J 2012; 33: 2451-96.
4. Malhotra A. The changing burden of valvular heart disease. BCS Editorial. 2012.