• Mitral stenosis usually results from rheumatic fever, but infants can be born with the condition.
• Mitral stenosis does not usually cause symptoms unless it is severe.
• Doctors make the diagnosis after hearing a characteristic heart murmur through a stethoscope placed over the heart.
• Treatment includes use of diuretics and beta-blockers or calcium channel blockers

In mitral stenosis, blood flow through the narrowed valve opening is reduced. As a result, the volume and pressure of blood in the left atrium increases, and the left atrium enlarges. The enlarged left atrium often beats rapidly in an irregular pattern (a disorder called atrial fibrillation). As a result, the heart's pumping efficiency is reduced. If mitral stenosis is severe, pressure increases in the blood vessels of the lungs, resulting in heart failure with fluid accumulation in the lungs and a low level of oxygen in the blood. If a woman with severe mitral stenosis becomes pregnant, heart failure may develop rapidly.

Cause

Mitral stenosis almost always results from rheumatic fever, a childhood illness that sometimes occurs after untreated strep throat or scarlet fever (see Bacterial Infections: Rheumatic Fever). Rheumatic fever is now rare in North America, Australasia, and Western Europe because antibiotics are widely used to treat infection. Thus, in these regions, mitral stenosis occurs mostly in older people who had rheumatic fever and who did not have the benefit of antibiotics during their youth or in people who have moved from regions where antibiotics are not widely used. In such regions, rheumatic fever is common, and it leads to mitral stenosis in adults, teenagers, and sometimes even children. Typically, when rheumatic fever is the cause of mitral stenosis, the mitral valve cusps are partially fused together.

Mitral stenosis can rarely be present at birth (congenital). Infants born with the disorder rarely live beyond age 2, unless they have surgery.

Three rare conditions unrelated to mitral stenosis can produce the same effects as the stenosis. They include a myxoma (a noncancerous tumor in the left atrium), cor triatriatum (a rare developmental abnormality in which a membrane goes across the left atrium), and pulmonary veno-occlusive disease (a narrowing of the veins that lead from the lungs into the left atrium).

Symptoms and Diagnosis

Mild mitral stenosis does not usually cause symptoms. Some people with more severe mitral stenosis have atrial fibrillation or heart failure. People with atrial fibrillation may feel palpitations (awareness of heartbeats). People with heart failure become easily fatigued and short of breath. Shortness of breath may occur only during physical activity at first, but later, it may occur even during rest. Some people can breathe comfortably only when they are propped up with pillows or sitting upright. Those people with a low level of oxygen in the blood and high blood pressure in the lungs may have a plum-colored flush in the cheeks (called mitral facies). People may cough up blood (hemoptysis) if the high pressure causes a vein or capillaries in the lungs to burst. The resulting bleeding into the lungs is usually slight, but if hemoptysis occurs, the person should be evaluated by a doctor promptly because hemoptysis indicates severe mitral stenosis or another serious problem.

With a stethoscope, doctors may hear the characteristic heart murmur as blood tries to pass through the narrowed valve opening from the left atrium into the left ventricle. Unlike a normal valve, which opens silently, the abnormal valve often makes a snapping sound as it opens to allow blood into the left ventricle. The diagnosis is usually confirmed by electrocardiography (ECG), a chest x-ray showing an enlarged atrium, and echocardiography, which uses ultrasound waves to produce an image of the narrowed valve and the blood passing through it.

Prevention and Treatment

Mitral stenosis will not occur if rheumatic fever is prevented by promptly treating strep throat with antibiotics. Treatment includes use of diuretics and beta-blockers or calcium channel blockers. Diuretics, which increase urine formation, can reduce blood pressure in the lungs by reducing the volume of circulating blood. Beta-blockers, and calcium channel blockers help control heart rhythms. Anticoagulants may be needed to prevent clot formation in people with atrial fibrillation.

If drug therapy does not reduce the symptoms satisfactorily, the valve may be repaired or replaced. Sometimes the valve can be stretched open using a procedure called balloon valvuloplasty. In this procedure, a balloon-tipped catheter is threaded through a vein and eventually into the heart. Once inside the valve, the balloon is inflated, separating the valve cusps. Alternatively, heart surgery may be performed to separate the fused cusps. If the valve is too badly damaged, it may be surgically replaced with an artificial valve.

People with mitral stenosis are given antibiotics before a surgical, dental, or medical procedure to reduce the small risk of developing a heart valve infection (infective endocarditis).