Rheumatic Heart Disease in Pakistan: Challenges and Opportunities

“Rheumatic fever licks at the joint, but bites at the heart.” Ernest-Charles Lasègue

Rheumatic heart disease (RHD) is a known complication of acute rheumatic fever (ARF), which is a sequel of untreated oropharyngitis infection caused by β-haemolytic group A Streptococcus pyogenes (GAS). RHD is the most common acquired heart disease in people aged under 25 years.^1,2 Globally, RHD affects an estimated 55 million people and results in approximately 360,000 deaths annually, the majority of which are premature deaths. According to research, the incidence of RHD is high in crowded areas with poor housing, bad sanitary conditions and lack of health literacy.³ All these factors contribute to higher reported numbers of cases of RHD in low- and middle-income countries (LMICs) or within marginalised populations in high-income countries, where compromised living conditions prevail.⁴ Fortunately, countries with a higher socio-demographic index have been successful in promoting health literacy, which has directly lowered the incidence and recurrence of ARF and therefore RHD.^5,6

Pakistan is the fifth most populous country in the world, with a population of >255 million.⁷ Pakistan’s health expenditure is significantly low, accounting for only about 1% of its gross domestic product, which is below the recommendations of the WHO. As a LMIC, Pakistan faces certain geopolitical and climate-change issues that have culminated in a higher incidence of RHD. Countries with a high socio-demographic index have adequately addressed these issues through improvements in healthcare systems, the widespread use of benzathine penicillin G (BPG) to treat streptococcal pharyngitis and the implementation of disease-control programmes.^8,9 These steps have not yet been taken in Pakistan due to a lack of sufficient resources and guidelines.¹⁰ Consequently, a large vacuum exists within the public health sphere that needs to be addressed.

Literature Search

A literature review was conducted based on an electronic search of the PubMed and Embase databases using keywords related to challenges and solutions for RHD in Pakistan up to 2025. This included the prevalence and incidence of RHD, challenges of RHD prevention (major challenges in RHD diagnosis and care include regional healthcare disparities, diagnostic limitations, scarcity of BPG for prophylaxis and lack of multidisciplinary RHD teams) in Pakistan and current landscapes of RHD prevention (Table 1). Evidence was derived from published observational studies, randomised clinical trials, systematic reviews and meta-analyses, reports and clinical guidelines. Studies that included Asian populations, were conducted in Asian countries, including Pakistan, and related to RHD prevention were considered for inclusion. Relevant international, regional and local clinical practice guidelines and society-related guidelines (e.g. from the American Heart Association and WHO) were reviewed. Reports from international and regional think tanks on RHD prevention efforts and challenges in Asian countries were also incorporated. Findings from preliminary searches were reviewed by the authors (FB, SS, DA, HI), with relevant information contained in this review. The final manuscript was then shared with all authors for editing and comments.

Understanding Acute Rheumatic Fever and Rheumatic Heart Disease

ARF is an autoimmune reaction that occurs following a throat infection caused by GAS. RHD develops as a chronic consequence of ARF, resulting from either one severe episode or repeated bouts of the illness that progressively damage the heart mediated by molecular mimicry, primarily affecting children aged 5–15 years.^11–14

Characteristic histological findings in rheumatic carditis include Aschoff’s bodies in the myocardium and MacCallum plaques on the valves and subendocardium of the left atrium. If left untreated, valvular damage can result in progressive heart failure or death.

Approximately 3–6% of individuals with GAS pharyngitis develop ARF, a proportion that remains consistent globally, even in regions where the disease is endemic.¹⁴ Around 60% of ARF cases progress to RHD within a decade, with recurrent ARF episodes worsening existing cardiac injury.¹⁵ Importantly, the development of ARF and RHD is more closely linked to access to timely diagnosis and treatment than to genetic susceptibility.¹⁶ In low-resource settings like Pakistan, socioeconomic challenges and policy gaps significantly contribute to the on-going burden and mortality associated with RHD.

Epidemiology

Annually, more than 5.5 million individuals are affected by RHD around the globe and approximately 360,000 lives are lost each year, with the large majority being in LMICs.¹⁷ Within this pool, the incidence of RHD in Pakistan is 8.0 per 1,000 people, with a predominance in women.^18,19 The incidence of RHD has shown an upward trend over the last 30 years, increasing from 883.00 per 100,000 individuals in 1990 to 991.01 per 100,000 by 2021. Alongside this rise, mortality rates remain a concern, with an estimated 17.85 deaths per 100,000 attributed to RHD in 2021.^11,14 This dramatic statistic makes Pakistan a country with the highest prevalence of RHD in all of South Asia.¹⁷ Even in children, the incidence of RHD is higher than congenital heart disease (3.5% versus 2.5%). In Pakistan, the population aged under 30 years is substantial, with approximately two-thirds of the population falling into this age group. Specifically, 67% of Pakistanis are aged under 30 years and 40.1% are aged under 15 years. This demographic trend positions Pakistan as a nation with a significant ‘youth bulge’. Unfortunately, this is also the age group particularly affected by RHD.

Significant discrepancies exist in RHD statistics from Pakistan, mainly as a result of limited access to diagnostics and the absence of robust epidemiological studies and patient registries. One study found that fewer than 20% of individuals with RHD were aware of their condition.^19–21 However, the introduction of rapid echocardiographic screening in smaller centres has improved detection of subclinical cases.¹² Despite data limitations, the persistently rising burden of RHD is evident and demands urgent public health attention.

Risk Factors for Rheumatic Heart Disease

RHD is significantly more prevalent in low-income countries and LMICs because of socioeconomic factors such as poverty, overcrowding, poor sanitation, malnutrition and limited access to healthcare.²² In Pakistan, 61% of urban and 85% of rural populations live in overcrowded conditions, and over 60 million people are illiterate, leading to poor hygiene practices and higher rates of streptococcal infections.^23,24

Malnutrition, particularly in children, increases vulnerability to GAS infections. In Pakistan, 10% of children under the age of 5 years are stunted and 17.7% are wasted.^25,26 A study from Lahore found 67% of 546 RHD-diagnosed children to be undernourished, indicating a link between malnutrition and disease severity.^27–29

Despite the global burden, no licensed GAS vaccine exists due to concerns over antigenic diversity and safety.³⁰ However, there is renewed focus on developing vaccines to prevent the initial infection and its autoimmune consequences.³¹

Group A Streptococcal Infections

GAS infections remain widespread in rural Pakistan because of sanitation issues, crowding and low awareness. Pharyngitis is often overlooked, leading to delayed treatment and antibiotic misuse. Diagnostic tools, such as rapid antigen detection tests and throat cultures, are underused, while antibiotic resistance and inadequate surveillance worsen outcomes. The absence of a prophylactic vaccine remains a significant barrier.

Acute Rheumatic Fever

ARF develops in 0.3–3% of GAS cases, mainly affecting children aged 5–14. In low-resource settings, lack of definitive diagnostic tests complicates detection. Although BPG prophylaxis reduces recurrence, barriers, such as high cost, limited supply, painful administration and workforce shortages, hinder its use.

Rheumatic Heart Disease

Without treatment, approximately 30–60% of ARF cases progress to RHD within a decade, especially if there is carditis during the initial episode, repeated ARF attacks and lack of secondary prophylaxis (i.e. monthly BPG injections). In Pakistan, patients typically present late due to the absence of early echocardiographic screening and insufficiently trained personnel. Management is further undermined by fragmented care, lack of multidisciplinary clinics and the absence of a centralised electronic health record system (Table 2).

Systemic Challenges

Limited Healthcare Access

More than half of Pakistan’s population lacks access to basic healthcare.¹⁶ With only six hospital beds per 10,000 people and frequent reliance on unregulated care, early intervention is often missed.³² While timely penicillin administration can reduce the risk of ARF by 80%, access to this treatment remains poor.³³

Lack of Education

In Pakistan, an estimated 22.8 million children are out of school, meaning they have never been enrolled in school or have dropped out, and will grow up illiterate.³⁴ This represents 44% of the total population of children aged 5–16 years. Consequently, running an effective school-based murmur detection service would not solve the issue because it would not reach the millions of children who do not attend school.

Workforce Shortages

The doctor:patient ratio of 1:1,300 in Pakistan is significantly lower than the WHO-recommended ratio of 1:1,000.³⁵ This ratio is reflected in a lower number of primary physicians and lack of specialists such as paediatricians and cardiologists, which results in delayed management of RHD and ARF. Most physicians serve urban areas; there is a dearth of physicians in rural areas.

As of July 2025, the Pakistan Medical and Dental Council reports 337,641 registered and licensed medical practitioners (167,319 men, 170,322 women); however, many specialists have migrated or are working abroad. With 40% of medical graduates leaving the country annually, the system is severely strained.³⁶ Although 170,322 female physicians are registered, a large number of them drop out due to poor recruitment and selection processes, transfer constraints, excessive workload with inadequate salary, harassment, sex discrimination, unsafe work environments and little support from the administration.^37,38 This further impacts the doctor:patient ratio. Moreover, only 140 cardiothoracic surgeons treat a significant burden of RHD and other cardiac ailments.^39,40 This critical shortage impacts diagnosis, treatment and outcomes.^41,42

Insufficient Funding

With healthcare spending at only $38 per capita (the WHO recommended spending is $44) and 2.6 million people living below the poverty line, financial barriers limit access to care.^43–46 Much healthcare spending is out-of-pocket. The insurance-based, government-run Sehat card scheme provided treatment costs for the underprivileged, but is now facing financial problems.

Lack of Awareness

Non-adherence to antibiotics because of early symptom relief and forgetfulness increases ARF risk and antibiotic resistance.^47,48 Measures such as public and provider education, and legislation to restrict the dispensing of antibiotics without a prescription are needed.

Lack of a Data Registry

The absence of a national RHD registry, combined with fragmented and methodologically weak hospital-based studies, hampers accurate burden estimation and effective policy responses.^24,49

Lack of a Structured Response

There is no tiered analytical framework to systematically address RHD-related challenges; diagnostic limitations, workforce shortages, and fragmented infrastructure remain unaddressed, resulting in poor long-term outcomes.

Delayed Presentation of Rheumatic Valvular Heart Disease Cases

Many patients fail to receive early care and proper management. These patients present with involvement of two or more heart valves, pulmonary hypertension, right heart failure, heart rhythm problems, thromboembolic complications, cardiac cachexia and endocarditis. These patients further burden the healthcare resources and impact outcomes.

Loss-to-follow-up and Non-adherence

In Pakistan, most of the population lives in rural areas. Patients diagnosed with ARF receive inadequate prophylaxis and hence progress to develop valvular involvement at an early age. The prophylactic monthly dose of deep intramuscular BPG injection is painful, so many patients become non-adherent. The over-the-counter availability of antibiotics without a prescription exacerbates the issue of antibiotic resistance. Patients who undergo an invasive valve procedure, such as percutaneous mitral balloon valvuloplasty or replacement, are less likely to attend follow-up clinics and are at a higher risk of developing other valvular lesions and heart rhythm problems. Patients with implanted mechanical heart valves and those with AF are poorly anticoagulated, with dire consequences of valve thrombosis, thrombo-embolic events and bleeding.

The Impact of Climate Change

Over the last decade, unsettled weather, massive floods and droughts have led to homelessness for millions of people. Temporary shelters, compromised living and make-shift arrangements with unhygienic waste disposal and sanitary conditions provide the ideal conditions for infectious diseases such as GAS to flourish and spread (Figure 1).

The Consequences of Rheumatic Heart Disease

The typical progression of valvular damage seen in ARF is mitral, followed by aortic, tricuspid then pulmonary valves.

Complications of RHD are vast and varied, including heart failure, AF, ischaemic embolic events and infective endocarditis.⁵⁰ As the leading cause of acquired heart diseases in both adolescents and children worldwide, RHD also causes mortality that is one of the highest in a few regions around the world including Pakistan.¹⁹ Moreover, RHD is the leading cause of premature death and disability in Pakistan.^50–52

According to a 2020 report by the WHO, 26,017 deaths due to RHD were reported from Pakistan. This is an estimated increase of 39.1%, rising from 15,199 in 1990 to 21,137 in 2019.^53,54 These statistics reflect a rapidly increasing trend, which can be extrapolated to estimate the burden of morbidity and mortality.

Nearly 45% of individuals with RHD are aged under 25 years. The majority require valvular surgery and, because of their young age at the time of surgery, many of these patients will require repeat interventions throughout their lives. Although valve repair is preferred when possible, mechanical valve replacement becomes necessary in a significant number of patients, including women of reproductive age. Lifelong anticoagulation in such cases introduces additional risk, particularly resulting from the difficulties of managing international normalised ratio levels in resource-limited settings.

An audit of indigenous Australian patients with RHD receiving warfarin revealed that, while 60% underwent international normalised ratio testing, only 25% had values within the recommended therapeutic range, increasing their vulnerability to bleeding or thromboembolic events.¹⁵

Opportunities for Improvement

Enhanced Awareness

One of the major barriers towards prevention of RHD in Pakistan is the lack of awareness.⁵⁴ By targeting specific misconceptions and treatment of ARF in the initial stages, the progression towards RHD can be curbed.^9,46 Furthermore, a study found that <20% of people were aware of their diagnosis. This highlights the need for awareness programmes in susceptible populations that will push them to seek help when they observe symptoms.^8,19 Similarly, healthcare professionals must also be trained to provide adequate risk assessment to their patients. They must provide proper counselling and disseminate preventative strategies that can be easily followed by at-risk populations. This is supported by research that claims that the successful elimination of RHD can occur based on comprehensive programmes.⁹

A systematic review by Tibazarwa et al. concluded that there are only a few population-based prospective studies that have been conducted around the world on the incidence of ARF.⁵⁵ With only 10 countries with population-based studies, even the most recent one was completed a decade ago. This gap underscores the need for current, robust and reliable data regarding ARF, especially in Pakistan, where research on heart diseases is already scarce. This information is essential for laying the foundations of governmental health policies and resource allocation that can combat RHD at a higher level.

Improved Access to Healthcare

Around 42% of the population in Pakistan does not have access to health insurance.⁵⁶ While changes to this can only be brought about on a national level, further comprehensive assessment of infrastructure, equipment and sterilisation techniques need to be carried out in public hospitals, which are the mainstay of treatment for a large population.^41,57

Disease Surveillance

Pakistan’s disease surveillance system must be revised to collect data for the monitoring of RHD; it is currently restricted to dengue, polio, hepatitis and tuberculosis.⁵⁸ Tools for the exchange of data and information must also be developed that will allow healthcare centres to share important data for the formation of a national registry. Currently, the lack of tools, differing data standards and poor data collection systems hinder the development of a national registry on RHD, which can be addressed through a multidisciplinary approach between various institutes.^55,59,60

Collaboration

Addressing the burden of RHD in Pakistan relies on several sectors beyond the healthcare system. An intersectoral collaboration between healthcare, education and social welfare departments is required. Immediate availability of medicines for GAS infection is crucial, alongside proper training of healthcare workers. The general population must also be educated through awareness sessions. Furthermore, it is important to understand that most people affected in Pakistan live in remote communities without access to primary healthcare. To reach this population, the aid of social workers will be required.

Limitations

This review is narrative and does not adhere to systematic review protocols. The synthesis of data was descriptive. Future efforts should focus on producing systematic, high-quality reviews.

Conclusion

RHD is a neglected yet serious burden on Pakistan’s healthcare system. It continues to place a significant burden on resources and public health setups alike. Much of the population is vulnerable, particularly children and young adults who are vital to the country’s future. The effect of this disease is not only visible through individual health outcomes but is also reflected in the form of long-term social and economic consequences, which disproportionately affect the most underserved communities. Thus, there is a pressing need to address the challenges posed by this endemic disease and to establish preventative strategies.

A comprehensive, multi-sector approach is essential to address this issue. Raising awareness about the incidence of RHD in rural communities and at schools, mechanisms for prevention, immediate diagnosis and treatment must also be developed. However, none of these interventions can be brought about without sufficient funding and governmental policies that aid in the swift execution of healthcare strategies.