The Persisting Burden of Rheumatic Heart Disease

Corresponding Author

Valvular heart disease (VHD) remains a leading cause of morbidity and mortality among elderly populations worldwide.¹ As global populations age and socioeconomic conditions evolve, the epidemiology, clinical characteristics, and management strategies for VHD have undergone significant shifts across diverse geographic regions.² In high-income nations, degenerative valve disease is becoming more prevalent, driven by increased life span and changes in the spectrum of VHD. Simultaneously, rapid advances in transcatheter valve interventions have revolutionized the therapeutic paradigm. In contrast, rheumatic heart disease (RHD) continues to be a predominant cause of VHD in low- and middle-income countries (LMICs), affecting approximately 41 million people globally, with Asia carrying a disproportionate burden.^3,4 Indonesia, an archipelagic nation characterized by geographical diversity and unequal access to health care services, represents a significant contributor to global RHD cases. Nevertheless, comprehensive nationwide data on the epidemiological and clinical management of RHD have been notably sparse. The Ina-RHD (Indonesia Rheumatic Heart Disease) multicenter study, featured in this issue of JACC: Asia, addresses this critical gap through a pioneering multicenter investigation, providing valuable insights into the clinical profile and management challenges of RHD in Indonesia.⁵

This study, conducted across 21 hospitals in 14 provinces, provides the first large-scale, systematically collected data on RHD in Indonesia. One of the most striking findings among the Ina-RHD study is the high proportion of female patients (64.4%), aligning with global patterns in RHD epidemiology.⁶ The predominance of mitral stenosis (MS)—especially isolated MS as the most common lesion (39.6%)—further reinforces the gendered nature of RHD, with mitral involvement strongly linked to atrial fibrillation (AF) and stroke. Notably, AF was observed in over 62.5% of patients, with even higher rates among those with mixed mitral valve lesions (77.3%) and isolated MS (70.4%). Moreover, the cohort’s clinical profile reveals late-stage disease at initial presentation: 25.1% were in NYHA functional class III-IV, 44.4% had a high probability of pulmonary hypertension, and 50% showed echocardiographic evidence of right heart involvement. These findings point to significant diagnostic and treatment delays, likely exacerbated by socioeconomic constraints, health care access disparities, and insufficient public awareness.

Stroke prevalence (6.6%) and a high burden of intracardiac thrombus (11.8% in AF patients) further underscore the urgency of appropriate anticoagulation. Yet, only 30.4% of patients on vitamin K antagonists (VKAs) achieved therapeutic International Normalized Ratio (INR) levels, and INR monitoring itself was suboptimal. The INVICTUS (The Investigation of Rheumatic AF Treatment Using Vitamin K Antagonists, Rivaroxaban or Aspirin Studies) trial showed that among RHD-associated AF patients, VKAs therapy led to a lower rate of a composite of cardiovascular events or death than rivaroxaban therapy, without a higher rate of bleeding.⁷ Therefore, VKAs remain the standard treatment, but their limitations have prompted researchers to explore solutions tailored to different cultural backgrounds, such as mobile health tools for tracking the INR or community anticoagulation clinics, and so on.

In addition, further improvements are still needed in the primary and secondary prevention of RHD. Only 37.3% of patients received secondary prophylaxis, and only 11.5% received intramuscular benzathine penicillin G—the gold standard for preventing recurrent acute rheumatic fever. Most relied on oral regimens, which are less effective and reflect barriers such as supply shortages, injection-related fear, and poor follow-up adherence. The identification of suboptimal anticoagulation and prophylaxis use is a clarion call for health system improvements, aligning with global calls for enhanced RHD control strategies.⁴

The Ina-RHD study highlights key priorities for addressing RHD in LMICs. Although global prevalence has declined, RHD continues to pose a significant public health challenge in endemic regions, where patients are often diagnosed at advanced stages—emphasizing the urgent need for early detection and consistent follow-up. Echocardiography-based screening is essential for identifying subclinical cases. Integrating RHD care into primary health systems can help close critical gaps in access and continuity of care.

In the era of artificial intelligence, diagnostic and therapeutic approaches must evolve accordingly. The artificial intelligence–assisted electrocardiogram interpretation and machine learning–enhanced chest x-ray analysis present promising tools for early detection in low-resource environments.8, 9, 10, 11 In parallel, the implementation of context-specific solutions—such as mobile health platforms and comprehensive education campaigns for patients and health care providers—is urgently needed to improve disease management. In terms of treatment options, transcatheter-based interventions may offer a valuable alternative for highly selected patients with RHD, particularly in regions where access to conventional surgical treatment is limited. Expanding access to such technologies could help bridge the gap between clinical needs and health care capacity in LMICs.

Despite its contributions, the study has limitations. Its hospital-based design may exclude patients with latent or undiagnosed RHD, potentially underestimating true prevalence. The reliance on retrospective medical records risks incomplete data, particularly for historical acute rheumatic fever, reported in only 6.7% of patients, which likely reflects diagnostic rather than epidemiological realities. The lack of longitudinal outcomes limits insights into disease progression, mortality, and intervention efficacy. Additionally, although the study identifies management gaps, it does not delve into root causes, such as patient education, provider training, or health care access barriers, which are critical for designing effective interventions.

In conclusion, the Ina-RHD registry marks a pivotal step in characterizing and addressing the burden of RHD in Indonesia. Its findings carry relevance for LMICs confronting similar epidemiological and health care challenges. By illuminating systemic gaps and demographic trends, this study reinforces the urgency of integrated, prevention-oriented, and technologically empowered strategies to mitigate the enduring threat of RHD. As global health priorities evolve, RHD must remain firmly on the radar—both as a marker of inequality and as a disease eminently preventable with the right interventions.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.