Elsevier

The Lancet

Volume 374, Issue 9697, 10–16 October 2009, Pages 1271-1283
The Lancet

Seminar
Mitral stenosis

https://doi.org/10.1016/S0140-6736(09)60994-6Get rights and content

Summary

Mitral stenosis is a common disease that causes substantial morbidity worldwide. The disease is most prevalent in developing countries, but is increasingly being identified in an atypical form in developed countries. All treatments that increase valve area improve morbidity. Mortality improves with surgery; the benefit of percutaneous balloon valvuloplasty to mortality might be similar to that of surgery but needs further study. Percutaneous balloon valvuloplasty is the treatment of choice for patients in whom treatment is indicated, except for those with suboptimum valve morphology, and even these patients are sometimes treated with this procedure if surgery is not feasible or if surgical risk is prohibitive. We review the pathology, diagnosis, and treatment options for patients with mitral stenosis.

Introduction

Although mitral stenosis is now rare in developed countries, it has been recognised for more than 300 years—Vieussens described the disease in 1705—and has provided major milestones in cardiology. It was the first disease to be diagnosed with echocardiography,1 and the first valve lesion to be successfully treated by surgery2 or percutaneous balloon valvuloplasty (PBV).3 Mitral stenosis remains an important cause of morbidity despite the ease with which it can be diagnosed and treated.

Mitrial stenosis is highly prevalent in developing countries4 because of its association with the prevalence of rheumatic fever, but is also seen in developed countries.5 Patients often have distinct social and demographic indicators dependent on their country of residence: in developing countries, patients tend to be young with a pliable valve,6 whereas in developed countries patients are of increased age with several comorbidities.7, 8

Section snippets

Epidemiology

Two-thirds of the world's population live in developing countries with a high prevalence of rheumatic fever or rheumatic heart disease (eg, 6 per 1000 schoolchildren in India vs 0·5 per 1000 in developed countries4), resulting in a large population with mitral stenosis. In a survey of rheumatic fever in India,4 the mean age of presentation was 15·1 years (SD 4·4), and two-thirds of the participants had signs of mitral stenosis, of whom half had limiting symptoms. Up to 30 million schoolchildren

Aetiology

Although mitral stenosis most frequently follows rheumatic fever, fewer than half of affected patients remember having rheumatic fever. Persistent inflammatory valve damage23 and haemodynamic injury contribute to gradual progression; the rate of progression and time to clinical detection is strongly associated with repeated episodes of rheumatic fever.24 This correlation could partly explain the different natural history of mitral stenosis across the world.10, 24, 25

Degenerative causes are

Pathology

The main features are leaflet thickening, nodularity, and commissural fusion, all of which result in narrowing of the valve to the shape of a fish mouth (figure 1).28 The leaflets might be calcified. Chordal fusion and shortening adds a further resistance to blood flow. Continued inflammation, injury, and repair affect the effectiveness of treatment, and treatments are tailored to target these features of disease.29, 30

Whether left-ventricular systolic function is truly reduced in patients with

Pathophysiology

The normal mitral valve area is 4–6 cm2 and a gradient is rare unless the valve is less than 2 cm2. Pathophysiology is closely related to the amount of diastolic flow across the valve and the diastolic filling period (Figure 2, Figure 3). Generally, symptoms of dyspnoea correlate with increasing mean left-atrial pressure, which is inversely related to RR interval (figure 3). Atrial contraction helps to maintain flow across the stenotic mitral valve; atrial fibrillation, which is associated with

Clinical presentation

Patients usually present with dyspnoea, often during exercise or in combination with disorders that increase heart rate or flow across the mitral valve.57, 58 The valve area narrows gradually by 0·1–0·3 cm2 per year,59 which explains the variable onset of symptoms. The disease course is accelerated in populations with recurrent rheumatic fever, in which the natural history is compressed to 5–10 years. Clinical presentation is affected by age-related comorbidities such as systemic hypertension,

Diagnosis

Physical signs of mitral stenosis have been well documented.58 Moderate-to-severe disease is easily diagnosed by auscultation in the left-lateral position or after mild exercise, unless the patient has unfavourable body habitus (severe obesity or chronic obstructive pulmonary disease), pulmonary oedema, very low cardiac output, or rapid atrial fibrillation. Diagnostic sensitivity and specificity is about 85% and accuracy is similar to that of echocardiography (92% vs 97%).62

Clinical issues that

Atrial fibrillation

Onset of atrial fibrillation, which is often caused by atrial inflammation and remodelling, is a pivotal moment in mitral stenosis. Atrial fibrillation occurs in 40–75% of patients who are symptomatic for mitral stenosis, precipitates such symptoms, greatly increases the risk of systemic embolisation, and reduces cardiac output and exercise capacity.77, 78 Exercise capacity is substantially improved by restoration of sinus rhythm,77, 78 especially in patients with small atria and short duration

Natural history and prognosis after intervention

The natural history of untreated57, 58, 105, 106 and treated disease19, 107, 108, 109, 110, 111, 112, 113, 114, 115, 116, 117, 118, 119, 120, 121, 122, 123, 124, 125, 126, 127 has been well characterised. Such studies have identified some key features about disease progression and treatment (Figure 5, Figure 6). First, progression in countries with recurrences of rheumatic fever tends to be rapid, whereas in those without rheumatic fever progression follows an indolent course. Second, all

Treatment

Treatment by drugs or techniques other than surgery or PBV for mitral stenosis is not very effective and therefore interventional treatment is preferred except in cases of a strong contraindication. Drugs can be used to slow heart rate, deal with atrial fibrillation, do gentle diuresis, and correct secondary conditions such as anaemia, fever, infection, and volume overload. β blockers or calcium channel blockers (eg, diltiazem) are the mainstay for control of heart rate; but β blockers might

Search strategy and selection criteria

We searched Medline for original articles published in English between January, 1970, and March, 2008, using the root search term “mitral stenosis”. We checked reference lists of selected articles for further relevant articles. Only directly relevant articles are included in the reference list of this report.

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