Acute rheumatic fever and rheumatic heart disease: Clinical sciences

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Introduction0:00–0:37

Acute rheumatic fever, or ARF for short, is a systemic inflammatory condition that develops after an infection with Group A Streptococcus, or GAS for short.
Acute rheumatic fever, which is thought to be an immune-mediated condition, affects the joints, skin, nervous system, and heart.
Moreover, if not recognized and treated on time, it can cause damage and scarring of the heart valves and structures. This condition is also known as rheumatic heart disease or RHD.
Now, if a pediatric patient is presenting with a chief concern suggesting ARF or RHD, first perform an ABCDE assessment to determine if they are unstable or stable.

Unstable patient0:37–1:12

If unstable, stabilize their airway, breathing, and circulation. Next, obtain IV access, consider starting IV fluids, and begin continuous vital sign monitoring, including blood pressure, heart rate, and pulse oximetry.
Finally, start broad-spectrum intravenous antibiotics, and, if needed, don’t forget to provide supplemental oxygen.Okay, now let’s go back to the ABCDE assessment and take a look at stable patients.

ARF1:12–1:52

Start by obtaining a focused history and physical exam. First, let’s discuss patients with ARF.
Your patient will likely report a history of fever and joint pain, as well as a recent history of infection characterized by a sore throat, with or without tonsillar exudates.
On the other hand, physical exam findings typically include joint tenderness, with or without effusion; a serpiginous rash; painless nodules on the shin; and even abnormal, uncontrolled movements.
In some patients, you might detect a heart murmur as well!These history and exam findings should make you suspect a recent GAS infection.

Suspect GAS infection1:52–3:25

Your next step is to obtain a rapid strep antigen test or a throat culture. Additionally, you should check strep antibodies, including antistreptolysin O; or anti-DNase B antibodies.
Finally, don’t forget to check the patient’s inflammatory markers, like ESR and CRP; and obtain an ECG and echocardiogram.Now, here’s a clinical pearl to keep in mind!
Strep antibody assays, like ASO and anti-DNase B, are often used to confirm the presence of a recent GAS infection, especially when a rapid antigen test or throat culture is negative.
However, these assays can be challenging to interpret, since antibodies linger in the body for months. So, you should not make decisions based on a single titer measurement!
Instead, follow the trend in your patient’s titers, because increasing titers are highly suggestive of recent GAS infection!Now, when it comes to labs, you may find that the rapid strep antigen test or throat culture is positive, or that your patient has detectable antibodies to strep.
In addition, the ESR and CRP are usually elevated. Finally, the ECG might reveal a prolonged PR interval, while the echo may show valvulitis or pericarditis.
At this point, you should suspect acute rheumatic fever. Once you suspect acute rheumatic fever, your next step is to assess for the JONES criteria, which consists of several major and minor criteria.

Acute Rheumatic Fever - JONES Criteria3:25–4:54

Major criteria include Joint involvement, more specifically migratory polyarthritis; Carditis, usually involving the left-sided heart valves, especially the mitral valve, or pericarditis; Nodules, which are subcutaneous and painless; Erythema marginatum; and finally, Sydenham chorea, which is a disorder associated with an involuntary, nonpurposeful movement.
Moreover, you can use the JONES mnemonic to remember the major criteria of acute rheumatic fever. On the other hand, the minor criteria include clinical features, such as fever and arthralgia; lab criteria, like elevated ESR or CRP; and ECG findings, such as a prolonged PR interval.
The JONES criteria are met when a patient fulfills at least 2 major criteria, OR 1 major criterion plus 2 minor criteria.
There is one exception to this; that is, the presence of Sydenham chorea alone is enough to diagnose ARF. Now, if JONES criteria are not met, you should consider an alternative diagnosis.
On the other hand, if JONES criteria are met, you can diagnose ARF.Now that you’ve diagnosed ARF, let’s move on to treatment.

Acute Rheumatic Fever- Treatment4:54–5:53

This starts with antibiotics and anti-inflammatory medications. Penicillins are the mainstay of antibiotic treatment, either in the form of oral amoxicillin; or oral or intramuscular penicillin.
On the other hand, the first-line treatment of symptomatic arthritis or carditis includes NSAIDs. If carditis does not respond to NSAIDs, or if they are contraindicated, you can consider aspirin or systemic corticosteroids.
Lastly, patients with acute rheumatic fever require secondary antibiotic prophylaxis, also known as long-term antibiotic prophylaxis, which helps reduce the risk of infection with new group A streptococcal strains!
This is critical, since GAS infections can trigger recurrent episodes of acute rheumatic fever and increase the risk of progression to rheumatic heart disease!
Speaking of which, let’s go back to our focused history and physical exam and discuss rheumatic heart disease. Your patient will likely report a history of previous GAS infection, such as strep pharyngitis, or a strep skin infection, like impetigo or erysipelas.

Rheumatic heart disease5:53–7:01

Additionally, they might have a known history of acute rheumatic fever or even describe symptoms of heart failure, such as lower extremity swelling, palpitations, fatigue, and shortness of breath that worsens with exercise.
On the other hand, important physical exam findings include heart murmur and lower extremity edema. Now, here’s a clinical pearl!
Even though rheumatic heart disease is a sequela of untreated acute rheumatic fever, the majority of patients actually present without a known history of acute rheumatic fever.
This suggests that, in many cases, symptoms are mild enough to be subclinical or not recognized at all. Additionally, keep in mind that these individuals often lack access to health care, such as immigrants from developing countries or uninsured individuals.Okay, at this point, you should suspect RHD, so your next step is to obtain a chest X-ray, an ECG, and a transthoracic echocardiogram.

Suspect RHD7:01–7:51

Alright, although it’s not diagnostic, use the chest X-ray to look for evidence of cardiomegaly or heart failure. So, you may see left atrial or left ventricular enlargement, and if the heart failure is severe, you may even see pulmonary congestion.
In addition, the ECG will reveal evidence of left atrial and left ventricular enlargement or even ventricular strain. Finally, the TTE might demonstrate mitral regurgitation; mixed mitral valve disease, like mitral regurgitation and mitral stenosis; or mitral and aortic valve disease.
With these findings, you can diagnose Rheumatic Heart Disease.Now, let’s discuss treatment for RHD. This includes medications used for heart failure, like diuretics and beta blockers; and if needed, secondary antibiotic prophylaxis.

Rheumatic heart disease - Treatment7:51–8:27

Additionally, consider consulting your surgery team for possible valve replacement! Here’s one last clinical pearl to keep in mind!
The valvular disease associated with rheumatic heart disease is often complicated by arrhythmias, such as atrial fibrillation, which predisposes the patient to thromboembolism.

Review8:27–9:24

Alright, as a quick recap… Acute rheumatic fever is an inflammatory condition following a GAS infection that affects the joints, skin, nervous system, and heart.
If you suspect this condition, start by evaluating for evidence of a prior GAS infection, and then use the JONES criteria to confirm your diagnosis.
Treatment includes penicillin, NSAIDs, and long-term antibiotic prophylaxis. On the other hand, rheumatic heart disease is a sequela of acute rheumatic fever characterized by cardiac damage and signs and symptoms of heart failure.
If you suspect rheumatic heart disease, order additional imaging to confirm the diagnosis. Treatment relies on heart failure medications; in some cases, secondary antibiotic prophylaxis; and, finally, surgical consultations for possible valve