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PH-CV13

Rheumatic fever and rheumatic heart disease

How an immune response to a streptococcal sore throat can inflame every layer of the heart weeks later, why the valves heal by scarring, and how repeated attacks fuse and narrow the mitral valve over years.

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  • 5 steps · 10 questions
  • 3D anatomy, affected parts marked

By the end, you can

  • Explain how molecular mimicry between streptococcal antigens and heart proteins causes carditis weeks after the infection.
  • Describe the pancarditis of acute rheumatic fever, including Aschoff bodies, Anitschkow cells and verrucae, and the other major manifestations.
  • Explain how healing and recurrences scar the valves, and why the mitral valve becomes a fish-mouth stenosis.
  • Relate mitral stenosis to left atrial pressure, lung congestion, atrial fibrillation and pulmonary hypertension, and to its heart sounds.
  • Apply the Jones criteria, recognise the classic appearances and tests, and explain how penicillin prophylaxis, anti-inflammatory drugs and valve procedures act on the mechanism.

What it is

Acute rheumatic fever is an inflammatory illness of the heart, joints, skin and brain that follows group A streptococcal infection, usually of the throat, after a delay of weeks; the tissues are not infected, and the damage comes from the immune response. Rheumatic heart disease is the lasting scarring of the heart valves, mostly the mitral valve, left by one or, more often, repeated attacks.

3 types to tell apart
Acute rheumatic fever
Weeks after the infection: carditis, migrating arthritis, chorea and skin signs, which settle over weeks to months.
Chronic rheumatic heart disease
Years to decades later: fibrosed, fused valves, causing mitral stenosis, mitral or aortic regurgitation, or a mixture, often in more than one valve.
Subclinical carditis
Valve inflammation or damage found by echocardiography without an audible murmur, in an acute attack or on screening where the disease is common.

US spelling: fibres → fibers.

Normal function

Thin valves that open wide and seal along a line

The heart wall has three layers: the endocardium, a thin lining of endothelium over connective tissue that also covers the valves; the myocardium, the muscle; and the pericardium, the serous sac around the heart. Each valve leaflet is a fold of endocardium around a core of dense collagen and elastic fibres. Healthy leaflets have almost no blood vessels: they are thin enough to be nourished by the blood flowing over them.

The mitral valve guards the inflow of the . Its and meet at two commissures, one at each end of the opening. In diastole they swing apart into the ventricle, leaving an orifice of about 4–5 cm², so blood crosses from the atrium with almost no pressure difference.

In systole the leaflets meet along a line of closure a few millimetres in from their free edges, held from turning inside out by that run to the . The left-sided valves work against the highest pressures in the heart, and the lines of closure are where the leaflets strike each other with every beat.

Point to it on the model