The primary health care (PHC) approach to rheumatic heart disease emphasises preventing initial episodes of rheumatic fever through primary prevention, avoiding recurrences by secondary prevention and ensuring long-term follow-up and an effective referral system. Primary care providers act as gatekeepers of community healthcare needs. They are heavily involved in community engagement, primary prevention, continuity of care and are part of strong PHC referral networks. This positions them ideally to participate in the prevention and early detection of rheumatic heart disease. The main aim of this CPD article is to guide the early identification and management of rheumatic heart disease within PHC settings.
Rheumatic heart disease (RHD) is a significant and increasingly prominent public health issue worldwide, affecting around 50 million people globally. The prevalence of RHD has increased 1.7 times since 1990.
The burden of RHD varies around the world. It has notably decreased in high-income countries because of improved sanitation, better housing, higher living standards and increased access to healthcare and antibiotics. In contrast, RHD mainly persists among rural, poor and marginalised communities with limited or no access to primary health care (PHC). The disease remains common in areas marked by poverty, overcrowding and healthcare inequalities.
Rheumatic heart disease remains the most common cause of acquired heart conditions among young adults in low- and middle-income countries (LMICs). Rheumatic heart disease accounts for an estimated 10.67 million disability-adjusted life years (DALYs) and causes around 360 000 deaths annually. This makes it the ninth leading cause of global mortality from invasive Group A Streptococcus (GAS) infections.
Rheumatic heart disease often causes serious complications, including infective endocarditis, heart failure, arrhythmias, pulmonary hypertension, systemic embolic events and stroke.
The impact of RHD has gained worldwide recognition, with the World Health Assembly (WHA) passing a historic resolution recognising it as a major global health concern. This resolution urges Member States to take vital actions, including strengthening multisectoral efforts to identify and prevent RHD at the PHC level.
Acute rheumatic fever is an autoimmune inflammatory reaction caused by GAS infection, and repeated episodes can lead to irreversible heart damage, as shown in
Natural history of acute rheumatic fever.
Many patients with ARF may experience subclinical or unnoticed initial events that lead to recurrent insults. The disease often remains undetected for years, with over 85% diagnosed only when significant heart damage occurs.
Recurrent and untreated streptococcal throat infections are the primary cause of rheumatic fever. Repeated episodes of rheumatic fever result in cumulative damage to the heart valves, especially the mitral and aortic valves.
Some individuals present a stronger autoimmune response to streptococcal antigens. Their immune system attacks cardiac tissue after reacting to streptococcal proteins.
Poor living conditions and overcrowding increase the risk of streptococcal infection transmission. Lack of sanitation and hygiene elevates the chance of recurrent infections.
Limited access to PHC, delayed diagnosis and absence of primary and secondary prophylaxis for GAS, including long-term penicillin prophylaxis after rheumatic fever. Lack of awareness among the public and PHC providers lead to under-diagnosis and inadequate follow-up. There is also a lack of prophylaxis to prevent the recurrence of rheumatic fever.
Children and adolescents are more vulnerable to ARF after initial GAS infection. Females may face a slightly higher risk, often because of interactions of the immune system. Some ethnic groups or indigenous populations, where GAS infections are endemic or healthcare access, is limited.
The Jones criteria are used to diagnose ARF and require evidence of a recent GAS infection, along with either two major manifestations or one major and two minor manifestations, as shown in
Low-risk populations: those with an incidence of ARF in children (5–14 years) < 2 per 100 000 per year or an RHD prevalence of ≤ 1 per 1000 population per year.
Moderate- or high-risk populations: those that have higher incidence and prevalence than the low-risk criteria.
Jones criteria for acute rheumatic fever.
| Low-risk population | High-risk population |
|---|---|
| Carditis (clinical or subclinical) | Carditis (clinical or subclinical) |
| Arthritis - only polyarthritis | Arthritis - monoarthritis or polyarthritis |
| Chorea | Polyarthralgia |
| Erythema marginatum | Chorea |
| Subcutaneous nodules | Erythema marginatum |
| Subcutaneous nodules | |
| Polyarthralgia | Monoarthralgia |
| Hyperpyrexia (≥ 38.5 °C) | Hyperpyrexia (≥ 38.5 °C) |
| ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl | ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl |
| Prolonged PR interval | Prolonged PR interval |
ESR, erythrocyte sedimentation rate; PR interval, start of the P wave to the beginning of the QRS complex interval.
Screening criteria for rheumatic heart disease.
| Valvular pathology in rheumatic heart disease |
|---|
| In individuals weighing < 30 kg: MR jet length ≥ 1.5 cm |
| In individuals weighing ≥ 30 kg: MR jet length ≥ 2.0 cm |
| MR jet is observed in at least one view |
| MR jet is observed in at least two consecutive frames |
| Any Aortic regurgitation |
| Observed in at least one view |
| Observed in at least two consecutive frames |
| Restricted leaflet motion with reduced valve opening |
MR, mitral regurgitation.
, Video link for parasternal long-axis view of mitral valve thickening and calcification:
Despite being the standard criteria, PHC providers often find the Jones criteria difficult to apply, which can lead to under-diagnosis of ARF,
Throat culture remains the gold standard for diagnosing pharyngitis caused by GAS, a precursor to ARF and RHD.
Serological tests to identify immune responses to bacterial enzymes (e.g. anti-streptolysin O, anti-DNase B, anti-hyaluronidase, anti-NADase and anti-streptokinase).
Molecular point-of-care (POC) testing.
Nucleic acid detection: real-time polymerase chain reaction (PCR).
Echocardiography is the most sensitive and gold standard method for diagnosing RHD owing to the following features:
Direct visualisation of valvular pathology.
Detection of subclinical carditis.
Integration of morphological and functional abnormalities.
Assessment of disease severity and physiological effects.
Identification of alternative diagnoses.
The WHO also recommends using point-of-care ultrasound (POCUS) to early identify rheumatic fever carditis and RHD in children, adolescents and adults in PHC settings where standard echocardiography is unavailable.
Prevention of RHD is conceptualised across four levels of care (see
Primordial prevention: aims to decrease GAS infections by enhancing living conditions and sanitation, as well as promoting community awareness and encouraging health-seeking behaviour. This serves as the fundamental strategy for eliminating RHD.
Primary prevention: involves treating GAS infections to prevent ARF and disease progression. In PHC settings where POC testing for GAS pharyngitis is unavailable, a clinical rule for suspected cases is recommended as a cost-effective approach to diagnosis.
Secondary prevention: seeks to prevent the recurrence of ARF through antibiotic prophylaxis to stop RHD development and progression. This is regarded as a fundamental part of RHD management.
Tertiary prevention: focuses on treating and managing RHD complications with medications for symptomatic relief and cardiac interventions, including surgery.
Stages of prevention of rheumatic heart disease.
Active case finding for RHD requires echocardiography to identify valvular changes in individuals without a history of ARF or RHD. This method can detect a variety of valvular alterations associated with different risks of disease progression. The screening criteria are designed to identify potential cases in PHC settings where RHD is common, and resources such as equipment and healthcare personnel are limited. They are intended for individuals aged 20 years or younger. Based on the best available evidence, these criteria aim to maximise sensitivity as a screening tool, even though this may lower specificity.
Management strategies for RHD encompass a multifaceted approach that includes eradication and prevention of GAS infection, symptomatic medical therapy, interventional procedures and surgery.
Long-acting penicillin remains the primary preventive measure. Benzathine penicillin G (BPG) every 3–4 weeks to prevent recurrent ARF episodes.
Patients with established, symptomatic moderate to severe valvular disease require pharmacological treatment primarily to relieve symptoms rather than to change outcomes.
Use loop diuretics and spironolactone and reduce afterload with vasodilator therapy (e.g. angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers). Digoxin and beta-blockers can also be considered based on individual patient needs.
Oral anticoagulation (warfarin) is essential for atrial fibrillation (AF) management and for patients with prosthetic valves. Novel oral anticoagulants (e.g. rivaroxaban, dabigatran) may be considered based on individual patient indications.
Percutaneous balloon valvuloplasty for patients experiencing symptoms; alternative options include cardiac surgery (valve repair or replacement).
Including good dental hygiene, vaccinations and pregnancy planning. Pregnant women with RHD require a multidisciplinary team comprising cardiologists, obstetricians and paediatricians.
Here are current challenges of RDH management in PHC:
Lack of awareness and low health literacy regarding RHD reported in a qualitative study from nine African countries.
Under diagnosis and lack of medical training among primary care providers.
Delayed diagnoses and treatment initiations in PHC settings.
Poor adherence to primary and secondary prophylaxis.
Scarcity of skilled health professionals.
There are opportunities in PHC for the management of RHD, which include:
Implementation of the echocardiographic screening tool and POCUS training within the PHC setting to identify children and adolescents at risk, particularly in endemic regions. Point-of-care ultrasound training should be integrated into the Bachelor of Medicine and Bachelor of Surgery (MBChB) curriculum and into additional training for the existing primary care workforce.
Early detection and prompt treatment of streptococcal pharyngitis with penicillin or appropriate antibiotics.
Integrated RHD follow-up care at primary care clinics ensures patients stick to their management plans.
Standardised protocols in primary care clinics for managing sore throats, rheumatic fever and suspected RHD.
Consolidate referral systems from primary care to specialised advanced care.
Community awareness campaigns on the importance of treating sore throats in school health programmes to identify children at risk.
Rehabilitation and supportive care for patients with established RHD who are not candidates for surgical interventions.
Integrating RHD programmes into wider PHC and NCD frameworks with comprehensive community-oriented primary care by multidisciplinary teams.
Rheumatic heart disease remains a neglected and preventable cardiovascular disease within unequal healthcare systems. Addressing RHD requires a multisectoral approach. Primary health care serves as the community’s first point of contact and is vital for RHD prevention through primary and secondary prophylaxis and early detection through ongoing screening. Managing RHD involves community education, easy access to care and appropriate referrals. The PHC approach has the potential to reduce the RHD burden, prevent complications and improve long-term outcomes. Primary health care is crucial to breaking this cycle, as RHD often results from untreated or recurrent Group A streptococcal infections.
The authors declare that they have no financial or personal relationships that may have inappropriately influenced them in writing this article. The author, Ramprakash Kaswa, serves as an editorial board member of this journal. The peer review process for this submission was handled independently, and the authors had no involvement in the editorial decision-making process for this article. The authors have no other competing interests to declare.
Ruchika Meel: Conceptualisation, Project administration, Writing – review & editing. Ramprakash Kaswa: Conceptualisation, Project administration, Writing – original draft, Writing – review & editing. Both authors reviewed the article, contributed to the discussion of results, approved the final version for submission and publication, and take responsibility for the integrity of its findings.
The authors confirm that the data supporting this study and its findings are available within the article and its listed references.
The views and opinions expressed in this article are those of the authors and are the product of professional research. It does not necessarily reflect the official policy or position of any affiliated institution, funder, agency or that of the publisher. The authors are responsible for this article’s findings and content.