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Rheumatic heart disease (PEN-Plus)

WHO RF/RHD guideline and PEN-Plus · reviewed October 2026 · gpreasoning.uk/global

Reasoning GP
Global Primary Care

Who / when

  • Children, adolescents and young adults with sore throat, joint pain with fever, jerky movements, or a heart murmur, in settings where RHD is common.
  • Anyone already diagnosed with rheumatic fever or RHD (for prophylaxis and follow-up).
  • Pregnant women with breathlessness, palpitations or a murmur.

Red flags: refer now

  • Breathless at rest or lying flat, swollen legs or abdomen: heart failure.
  • Irregular, fast pulse; fainting.
  • Sudden weakness, speech difficulty or facial droop (stroke from a clot).
  • Fever with a known valve lesion and no clear cause (possible endocarditis).
  • Pregnancy with known RHD, or with breathlessness and a murmur.
Heart failure, arrhythmia, stroke or pregnancyRefer the same day for assessment at a hospital with echocardiography and specialist support.

Assess

Sore throat

WHO could not recommend a clinical scoring rule that reliably identifies strep throat. Use a rapid or laboratory test where available.

Suspected rheumatic fever: revised Jones criteria

Evidence of a recent group A strep infection plus 2 major, or 1 major and 2 minor, manifestations (recurrence: 2 major, 1 major and 2 minor, or 3 minor).

Major (moderate- and high-risk settings)Minor (moderate- and high-risk settings)
Carditis (clinical, or valve inflammation on echo)Pain or swelling in one joint
Arthritis in one or more joints, or pain in several jointsFever 38.5 °C or more
Chorea (jerky, involuntary movements)ESR 30 mm/h or more and/or CRP 3.0 mg/dL or more
Erythema marginatum; subcutaneous nodulesProlonged PR interval on ECG (unless carditis is counted)

Rheumatic heart disease

Listen for a murmur. Diagnosis needs echocardiography; handheld echo can be used where standard echo is not available.

Classify

PictureClassify asAction
Known or suspected RHD with heart failure, irregular pulse, stroke signs, or in pregnancyComplicated RHDRefer the same day
Jones criteria met, or murmur with a history of rheumatic feverRheumatic fever or suspected RHDRefer for echo and specialist review; start prophylaxis once diagnosed
Sore throat, strep test positive, or no test available in a moderate- or high-risk setting (child or adolescent)Strep throat (confirmed or suspected)Penicillin
Diagnosed RF or RHD, stableOn secondary prophylaxisBenzathine benzylpenicillin every 4 weeks; regular review

Treat

Sore throat (primary prevention)

  • Treat with antibiotics if the strep test is positive (any age).
  • Where RF and RHD are common and no test is available: treat children and adolescents with suspected strep throat.
  • First line: penicillin, oral or intramuscular. Dose and duration per national formulary.

Secondary prophylaxis (prevents recurrence and progression)

  • Everyone diagnosed with rheumatic fever or RHD should be on prophylaxis. Children and adolescents with RHD found on echo screening should be too.
  • Preferred: benzathine benzylpenicillin (BPG) by intramuscular injection once every 4 weeks. Dose and total duration per national guideline.
  • If BPG cannot be used: oral penicillin is acceptable.
  • Mild past penicillin allergy (no anaphylaxis, angioedema or severe skin reaction): an oral penicillin test dose may be given before BPG. No allergy testing is needed for people with no history of allergy.
  • A local anaesthetic may be mixed with BPG to reduce injection pain.
Safe injectionsHave adrenaline ready wherever BPG is given, and know how to treat anaphylaxis; serious reactions are uncommon but do happen.

Follow-up

  • Keep a register of everyone on prophylaxis and the due date of each injection; trace anyone who misses one.
  • Each visit: breathlessness, exercise tolerance, palpitations, pulse rhythm, swelling, pregnancy plans.
  • Specialist or PEN-Plus clinic review with echo at intervals set by the team.

Counsel

  • Sore throats can lead to heart damage: come for treatment and finish the course.
  • The monthly injection stops new attacks that damage the heart further. Missing injections is risky.
  • Come at once for breathlessness, swelling, palpitations or fainting.
  • Women with RHD: plan pregnancies with your clinic; pregnancy strains the heart.

Your heart valves were damaged by rheumatic fever. The injection every 4 weeks protects them from more damage, so please never miss it.

When to refer

  • Same day: any red flag.
  • Suspected rheumatic fever or new murmur: for echo and confirmation.
  • Known RHD needing heart failure treatment, rhythm control or anticoagulation (PEN-Plus clinic).
  • Pregnant or planning pregnancy with RHD.

Sources

World Health Organization. WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease. 2024. WHO Regional Office for Africa. PEN-Plus: a regional strategy to address severe noncommunicable diseases at first-level referral health facilities (AFR/RC72/4). 2022. World Health Organization. WHO Model List of Essential Medicines. 2025.
Adapted from WHO guidance; not endorsed by WHO. This page paraphrases and adapts the WHO publications listed under Sources, which are licensed CC BY-NC-SA 3.0 IGO; the adapted text is shared under the same licence for non-commercial education. Reasoning GP is not affiliated with WHO or any other organisation named here. Use it alongside your national guidelines, essential medicines list and formulary, and your clinical judgement. Doses are given only where they come from a WHO source; otherwise follow your national guideline or formulary.