Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

non-pregnant

1st line – no therapy required

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ONGOING
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non-pregnant
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progressive asymptomatic disease (gradient <5 mmHg, valve area >1.5 cm²)
1st line – 

no therapy required

Patients with less than severe disease do not benefit from mechanical therapy, because it is not likely to significantly increase an already relatively adequate valve area.

1st line – no therapy generally required

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ONGOING
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non-pregnant
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severe asymptomatic disease (gradient >5 mmHg, valve area <1.5 cm²)
1st line – 

no therapy generally required

Asymptomatic patients with severe disease usually do not require therapy.

Consider – balloon valvotomy, valve replacement or repair

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ONGOING
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non-pregnant
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severe asymptomatic disease (gradient >5 mmHg, valve area <1.5 cm²)
Consider – 

balloon valvotomy, valve replacement or repair

Additional treatment recommended for SOME patients in selected patient group

Balloon valvotomy for patients with rheumatic disease may be considered for asymptomatic patients with very severe disease (valve area <1.0 cm²) when valve morphology is favourable or when atrial fibrillation intervenes. The presence of pulmonary hypertension may also be considered in the decision to proceed to balloon valvotomy.

Balloon valvotomy is not an appropriate treatment option for patients with mitral annular calcification (MAC)-related disease. In patients with MAC, balloon valvotomy is ineffective because the restriction to flow resides not in the valve itself, but rather, in dense calcification of the annulus which is resistant to dilation. Surgical valve replacement has a poor outcome due to the risk atrioventricular disruption when the calcium in the annulus is removed to permit placement of a valvular prosthesis.[40]​ In one study, the median 30-day surgical mortality was 6.3%, but was as high as 27%.[41] Further, most patients with MAC are older people and have multiple comorbidities, additionally worsening prognosis. In general, if such patients are considered for surgery, they should be referred to surgeons with a high volume experience in treating MAC-related disease.​

1st line – diuretic

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ONGOING
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non-pregnant
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severe symptomatic disease (gradient >5 mmHg, valve area <1.5 cm²)
1st line – 

diuretic

A diuretic may reduce left atrial pressure and relieve mild symptoms, although diuretic therapy alone is rarely adequate to relieve symptoms. Mechanical relief from valve obstruction is often the only effective remedy for severe symptoms and for pulmonary hypertension.

Primary options

furosemide: 40 mg orally once daily initially, titrate dose according to response, maximum 600 mg/day

OR

bumetanide: 0.5 mg orally once daily initially, titrate dose according to response, maximum 10 mg/day

Plus – balloon valvotomy, valve replacement or repair

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ONGOING
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non-pregnant
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severe symptomatic disease (gradient >5 mmHg, valve area <1.5 cm²)
Plus – 

balloon valvotomy, valve replacement or repair

Treatment recommended for ALL patients in selected patient group

Mechanical relief from valve obstruction is often the only effective remedy for severe symptoms and for pulmonary hypertension in patients with rheumatic disease. Percutaneous balloon valvotomy is the treatment of choice when valve anatomy is suitable for this procedure. Each of the following is graded on a severity score of 1 to 4: valve mobility, calcification, leaflet thickening, and sub-valvular apparatus distortion. Thus a minimum score of 4 and a maximum of 16 are possible. Valves with a score of less than 9 are usually considered optimal for balloon valvotomy. In patients with higher valve scores, surgery to perform open valve commissurotomy or valve replacement is undertaken.

Balloon valvotomy is not an appropriate treatment option for patients with mitral annular calcification (MAC)-related disease. In patients with MAC, balloon valvotomy is ineffective because the restriction to flow resides not in the valve itself, but rather, in dense calcification of the annulus which is resistant to dilation. Surgical valve replacement, has a poor outcome due to the risk atrioventricular disruption when the calcium in the annulus is removed to permit placement of a valvular prosthesis.[40] In one study, the median 30-day surgical mortality was 6.3%, but was as high as 27%.[41] Further, most patients with MAC are older people and have multiple comorbidities, additionally worsening prognosis. In general, if such patients are considered for surgery, they should be referred to surgeons with a high volume experience in treating MAC-related disease.​​ 

Consider – anticoagulation

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ONGOING
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non-pregnant
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severe symptomatic disease (gradient >5 mmHg, valve area <1.5 cm²)
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Patients undergoing valve replacement require anticoagulation. A vitamin K antagonist (e.g., warfarin) is recommended in patients with rheumatic disease.[22] In patients with mitral annular calcification (MAC)-related disease, direct oral anticoagulants (DOACs) may be suitable for atrial fibrillation in these patients. However, no studies exist to compare vitamin K antagonists to DOACs in MAC-related disease. Consult your local protocols for further guidance on suitable anticoagulation options and doses.

Bleeding complications from the anticoagulants used to prevent thromboembolism occur approximately at a rate of 2% per year.[34]

Consider – beta-blocker or ivabradine

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ONGOING
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non-pregnant
|
severe symptomatic disease (gradient >5 mmHg, valve area <1.5 cm²)
Consider – 

beta-blocker or ivabradine

Additional treatment recommended for SOME patients in selected patient group

For patients with mitral stenosis in normal sinus rhythm who experience worsening of symptoms with exercise, heart rate control with a beta-blocker may be considered.[22]​​

Ivabradine may be an alternative to beta-blockers for rate control in these patients. Three trials compared beta-blockers and ivabradine for exercise tolerance in patients with severe mitral stenosis.[37][38][39] In all three trials, ivabradine improved exercise tolerance compared with baseline and in two of the trials, ivabradine was superior to beta blockade.[37][38]

Primary options

atenolol: 25-100 mg orally once daily

OR

ivabradine: 2.5 to 5 mg orally twice daily initially, titrate dose according to response and resting heart rate, maximum 15 mg/day

pregnant

1st line – diuretic

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ONGOING
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pregnant
1st line – 

diuretic

Pregnant women with mitral stenosis may develop symptoms in the second trimester, when the demand for cardiac output increases by around 70%. A diuretic is given to control mild symptoms.

A systematic review found a lack of evidence concerning effectiveness of the various treatments for valvular heart disease in pregnancy.[42]

Primary options

furosemide: 40 mg orally once daily initially, titrate dose according to response, maximum 600 mg/day

OR

bumetanide: 0.5 mg orally once daily initially, titrate dose according to response, maximum 10 mg/day

Consider – balloon valvotomy

Back
ONGOING
|
pregnant
Consider – 

balloon valvotomy

Additional treatment recommended for SOME patients in selected patient group

If symptoms worsen despite diuretics and heart failure threatens the life of mother or child, balloon valvotomy can be performed safely in patients with rheumatic disease.

A systematic review found a lack of evidence concerning effectiveness of the various treatments for valvular heart disease in pregnancy.[42]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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