Rheumatic heart disease is a preventable yet serious public health problem in low- and middle-income countries and in marginalized communities in high-income countries, including indigenous populations.
The disease results from damage to heart valves caused by one or several episodes of rheumatic fever, an autoimmune inflammatory reaction to throat infection caused by Group A β-Hemolytic Streptococcus (GAS). It most commonly occurs in childhood, and can lead to death or life-long disability. Effective early intervention can prevent premature mortality from rheumatic heart disease.
Rheumatic heart disease disproportionately affects girls and women.
The risk of developing rheumatic heart disease is up to two times higher for females than males, and females accounted for two thirds of patients with rheumatic heart disease admitted to selected hospitals in 12 countries in the African Region, India and Yemen. Where rheumatic fever and rheumatic heart disease are endemic, rheumatic heart disease is the principal heart disease seen in pregnant women, causing significant maternal and perinatal morbidity and mortality. (WHO, 2018)
The diagnosis of ARF is not based on a single definitive test but on a combination of clinical and laboratory findings that satisfy the classification criteria known as the Jones Criteria. A patient is said to have ARF if there is the presence of two major manifestations or one major and two minor manifestations. It should be noted that these are classification criteria, not diagnostic criteria, so clinical judgment is paramount.
1. The major manifestations of the Jones criteria can be remembered by using the mnemonic JONES = J-O-N-E-S and imagining a heart shape in place of the O! J = migratory polyarthritis (J stands for joints!) O = carditis (O shaped like a heart!) N = subcutaneous nodules (N stands for Nodules!) E = erythema marginatum (rash) (E stands for Erythema!) S = Sydenham chorea (S stands for Sydenham!)
2. The minor manifestations of the Jones criteria include fever, arthralgia, elevated acute phase reactants (ESR/CRP), and a prolonged PR interval on ECG.
3. Supporting evidence of an antecedent Group A β-Hemolytic Streptococcus (GAS) infection can be obtained with elevated streptococcal antibody titres (anti-streptolysin or anti-deoxyribonuclease B) or a positive throat swab.
4. Joint (arthritis or arthralgias) and cardiac (carditis or prolonged PR interval) manifestations can only be counted once as either a major or minor criterion but not both.
Now that we have established the criteria for the diagnosis of ARF, let’s review how ARF would typically present in an outpatient setting. The onset of ARF occurs 2-3 weeks following an initial tonsillopharyngeal infection with group A β hemolytic Streptococcus. After an asymptomatic interim period, the onset of ARF is often accompanied by minor manifestations of the disease including fever, arthralgias, elevated acute phase reactants, and a prolonged PR interval on ECG. These are non-specific and can be found in multiple other conditions. The severity and duration of the manifestations vary from one individual to another.
Arthritis is the most common of the major manifestations, occurring in 70% of patients with ARF. The arthritis primarily affects large joints, such as the knees and ankles, and is different from the arthritis in most other rheumatologic diseases in that it is migratory in nature. It often comes and goes and will involve different joints over time. The arthritis of ARF responds well to treatment with ASA or other NSAIDs.
Carditis, the affected leaflet is the endocardium in more than 90% of the cases, which is expressed as mitral regurgitation, manifesting as an apical systolic murmur.
In approximately 50% of the cases, it may be accompanied by basal diastolic murmur, due to aortic regurgitation. The concomitance of mitral and aortic regurgitation in a previously healthy patient is highly suggestive of rheumatic fever. Occasionally, myocarditis and pericarditis may be present. In the absence of valvulitis, these manifestations are rare in rheumatic fever
Chorea – disordered, involuntary, abrupt movements of skeletal striated muscle groups. Complaints include stumbling during ambulation, slurred speech, dropping or throwing objects such as dishes, cups, notebooks, and bad calligraphy.
It affects more females than males, in the adolescent age group. There is considerable emotional liability, easily alternating between crying and laughter. The differential diagnosis with systemic lupus erythematosusis necessary, especially in cases of difficult therapeutic control.
Erythema marginatum and subcutaneous nodules – are rare but highly specific to rheumatic fever. Erythema marginatum is a pink macular lesion with a rounded margin and pale center. It is usually not pruriginous and spares the face. The subcutaneous nodules are painless and are usually located on the extensor surfaces of the joints and along the tendons. They are associated with the presence of carditis
The first therapeutic measure is the eradication of the causative infectious agent, the group A β hemolytic Streptococcus: penicillin G benzathine, IM, 1,200,000 U, for children weighing more than 20 kg; 600,000 U for children weighing up to 20 kg.
• For patients with hemorrhagic disorders (that cannot receive medication through IM route) penicillin-V, orally (50 mg/kg/day, 4 times daily) or amoxicillin (50 mg/kg/day, taken three times daily), both for 10 days.
• For atopic patients, allergic to penicillin and derivatives: erythromycin (40 mg/kg/day, four times daily for 10 days) or azithromycin (20 mg/kg/day, once daily for 3 days). Tetracycline (high prevalence of resistance), sulfonamides (do not eradicate the agent), chloramphenicol (high toxicity) should not be used.
• Arthritis: non-steroidal anti-inflammatory drugs for about 7–10 days, preferably orally: Acetyl-salicylic acid (80–100 mg/kg/day); Naproxen (10–20 mg/kg/day); Ibuprofen (30–40 mg/kg/day); Ketoprofen (1.5 mg/kg/day)
• Carditis: prednisone (1–2 mg/kg/day), orally, maximum of 60 mg/day. Use full dose, fractionated into 2 or 3 daily doses for 15 days; then reduce 20–25% of the dose per week.
Note: in case of concomitant arthritis and carditis, there is no need to use non-steroidal anti-inflammatory drugs; when promoting the gradual reduction of prednisone, it is not necessary to introduce non-steroidal anti-inflammatory drugs, providing that a weekly reduction higher than 25% is not intended.
• Chorea: haloperidol, orally, at a dose of 1 mg/day (and not per kg) twice daily. Increase 0.5 mg every 3 days until a good response is attained (more than 75% remission of the movements) or up to a maximum dose of 5 mg/day.
Treatment duration of three months.
Note: Doses close to the maximum dose may cause impregnation or extrapyramidal syndrome. Valproic acid (30 mg/kg/day, orally, starting with 10 mg/kg/day and increasing 10 mg/kg, weekly) is indicated as an alternative.