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Rheumatic Heart Disease: Important Steps to Tackle a Misunderstood Disease

Wednesday, February 22, 2023

Vervoort D. Rheumatic Heart Disease: Important Steps to Tackle a Misunderstood Disease. February 2023. doi:10.25373/ctsnet.22151729.v1

Valvular heart disease (VHD) is a leading and growing form of cardiovascular disease worldwide. VHD presents in many forms, but its epidemiology is predominantly driven by chronic noncommunicable disease origins—calcific aortic stenosis and degenerative mitral valve disease—and chronic sequelae from acute communicable disease origins—rheumatic heart disease (RHD) following untreated acute rheumatic fever (1). 

Whereas calcific aortic stenosis (12.6 million people) and degenerative mitral valve disease (18.1 million people) are some of the leading causes of cardiovascular disease worldwide, RHD remains the most common form of VHD, with more than 40 million people living with RHD to date (2–5). Shockingly, this number is likely far higher in reality (6). 

The early detection and antibiotic treatment of streptococcal infection and acute rheumatic fever have resulted in reductions in RHD burdens in high-income countries, where RHD is currently only seen in older patients (i.e., sequelae from past infections), marginalized populations (e.g., homeless individuals), immigrant communities, remote areas, and among Indigenous Peoples (7,8). This trend in the global incidence and prevalence, which shifted to predominantly low- and middle-income country (LMIC) populations, reduced global interest and action to address RHD. 

Today, RHD is considered “neglected among the neglected” because of its disproportionate underfunding relative to its disease burden, whereby most other “neglected tropical diseases” are receiving more funding, policy attention, and collective effort despite lower disease burdens (9). 

This article serves as an overview of landmark policy documents and societal efforts to address RHD and concludes with a call to action for the cardiothoracic surgery community to accelerate efforts and leave no patient with RHD behind.

Addis Ababa Communiqué

In 2008, cardiologists and cardiac surgeons convened in Drakensberg, South Africa, to develop the Drakensberg Declaration on the Control of Rheumatic Fever and Rheumatic Heart Disease in Africa, which served as a first collective call to action from the cardiovascular community to address the global burden of RHD (11). Several years later, in 2015, healthcare professionals, researchers, and policymakers from across Africa were convened by the Pan-African Society of Cardiology (PASCAR) in Addis Ababa, Ethiopia. In consultation with the Social Cluster of the Africa Union Commission, they set out to develop a roadmap addressing the burden of RHD on the continent, titled the “Addis Ababa Communiqué on Eradication of Rheumatic Heart Disease in Africa” (12). The roadmap laid out seven key steps (13):

1.    Establish prospective registries for RHD at sentinel sites.

2.    Attain and maintain adequate supplies of antibiotics (i.e., benzathine penicillin) for the primary and secondary prevention of acute rheumatic fever and RHD.

3.    Improve access to reproductive health services for women with RHD and other noncommunicable diseases.

4.    Decentralize the expertise and technology (e.g., echocardiogram) for the diagnosis and management of acute rheumatic fever and RHD.

5.    Establish national and regional centers of excellence for essential cardiac surgery and cardiac surgical training.

6.    Develop and embed national, multisectoral, and multidisciplinary RHD programs within existing or budding noncommunicable disease control programs.

7.    Foster international partnerships for resource mobilization, monitoring, and evaluation.

The Communiqué was soon endorsed by the Heads of State of African Union members and served as a strong political statement that RHD requires greater priority on global and national health agendas. However, inconsistencies in civil society messaging, underfunding, limited research, and competing political priorities resulted in insufficient action in recent years to meet the Communiqué’s goal of “a 25% reduction in mortality from RHD by the year 2025.” (12, 14–16). 

The Cape Town Declaration on Access to Cardiac Surgery in the Developing World

In 2017, the world celebrated fifty years since the first heart transplantation by Dr. Christiaan Barnard at Groote Schuur Hospital in Cape Town, South Africa. To honor this landmark moment in the history of cardiac surgery, representatives from the major cardiac surgical societies and the World Heart Federation convened in Cape Town, South Africa, to tackle one of the most pressing cardiac surgical burdens today: RHD. The representatives came to a consensus and issued the Cape Town Declaration on Access to Cardiac Surgery, which primarily focused on RHD, with the following aims (17):

1.    To establish an international working group (coalition) of individuals from cardiac surgery societies and representatives from industry, cardiology, and government to evaluate and endorse the development of cardiac care in low- to middle-income countries.

2.    To advocate for the training of cardiac surgeons and other key specialized caregivers at identified and endorsed centers in low- to middle-income countries.

The first aim was accomplished by the development of the Cardiac Surgery Intersociety Alliance, a collaborative effort between the Society of Thoracic Surgeons (STS), the American Association for Thoracic Surgery (AATS), the Asian Society for Cardiovascular and Thoracic Surgery (ASCVTS), the European Association for Cardio-Thoracic Surgery (EACTS), and the World Heart Federation (18). The foundation for the second aim has been laid by the identification of two hospitals in LMICs, the Maputo Heart Centre in Mozambique and King Faisal Hospital in Rwanda, to support the expansion of these centers into regional hubs and future training centers for cardiac surgery (19). 

World Health Assembly Resolution

Efforts to address the global burden of RHD did not stop at the continental and societal level. In 2018, the World Health Assembly—the annual convening of the World Health Organization, Ministries of Health, and other governmental, intergovernmental, and non-governmental organizations—adopted the Resolution WHA 71.14 on Rheumatic Fever and Rheumatic Heart Disease (20). The Resolution was endorsed by all Ministries of Health and served as another strong political statement and the foundation for the development of a coordinated global response to tackle acute rheumatic fever and RHD. Unfortunately, the Resolution did not address the need for cardiac surgical care for the dozens of millions of people already living with RHD and, beyond this single Resolution, little country-level action has been taken to address the global burden of RHD (15, 21). 

Call to Action

The gaps in care remain enormous, and further action should and can be taken by the cardiothoracic surgery community immediately, including the following nonexhaustive list:

1.    Advocacy (22): Awareness must be raised about the dreadful state of care for RHD worldwide. Everyone can inform themselves about the numbers and policies described above and pass on the information to colleagues, peers, mentors, mentees, and social media communities (23). However, consistency in messaging is equally necessary (16). Through consensus by a multidisciplinary and global group of experts and stakeholders, the World Heart Federation has developed a roadmap to tackle RHD, serving as a "global [strategy] designed to help governments, employers, nongovernmental organizations (NGOs), health activists, academic and research institutions, health care providers and people who have been affected by [RHD], take action to better prevent and control [RHD]” (24). Similarly, the next generation of cardiovascular care specialists and research should be engaged to ensure that health system interventions planned for the future involve those who will implement and follow up on those policy plans. For example, the Global Cardiac Surgery Initiative is a global network of trainees and young surgeons advocating for the six billion people without access to safe, timely, and affordable cardiac surgical care when needed (25). 

2.    Collaboration: RHD is a complex condition involving all levels of prevention and care as well as the wide spectrum of social determinants of health. As such, it is paramount that efforts to address RHD involve multisectoral and multidisciplinary stakeholders. Moreover, patients must be centered in discussions and interventions surrounding RHD. For example, the Global Alliance for Rheumatic and Congenital Hearts (Global ARCH) is a global alliance of more than fifty CHD and RHD patient-family groups around the world, of which approximately half are based in LMICs.

3.    Equity: RHD is a disease of poverty, mostly affecting populations in LMICs and select marginalized populations in high-income countries. In contrast, cardiovascular diseases—and noncommunicable diseases at large—are often falsely perceived as issues mostly affecting high-income countries (26). The Lancet Commission on NCDs and Injuries (NCDIs) Among the Poorest Billion recognizes that current global health agendas, especially those pertaining to noncommunicable diseases such as cardiovascular disease, do not adequately represent and address the needs of the world's poor (27). Tackling RHD will require an equity-first agenda, whereby cardiac surgery must no longer be considered a luxury only for those able to pay.

4.    Funding: Funding for both noncommunicable diseases (less than 2 percent of global health funding) and global surgical care (approximately 1 percent) is disproportionately limited (28,29). Specifically, RHD receives a very minor portion of global health funding, whereas the portion of funds dedicated to global cardiac surgical care is undefined (9). Funding must be increased through a variety of sources, including the use of novel and innovative financing instruments, and apply a health systems scope that may benefit both the prevention and management of acute rheumatic fever and RHD (30,31). Existing funding mechanisms, such as the Thoracic Surgery Foundation Every Heartbeat Matters Award, supported by the Edwards Lifesciences Foundation, must also be maintained and applauded.

5.    Policy: There has been a historical gap between health policy and healthcare. Policymakers are often not rooted in healthcare, whereas healthcare professionals are rarely involved with policy processes. As a result, cardiac surgeons, cardiologists, and other members of the cardiovascular care team are generally not “at the table” when matters pertaining to cardiovascular care or health systems strengthening are discussed (22). This is likely one reason why cardiac surgery is almost consistently missing from international health policy and global surgical policy, such as National Surgical, Obstetric, and Anesthesia Plans (21,32). Health policy engagement can be pursued individually as well as through professional societies, such as the Society of Thoracic Surgeons’ PAC, which provides a voice and opportunity for engagement for the cardiothoracic surgery community in United States policymaking.

6.    Research: Despite a dearth of research on RHD, cardiac surgical and global cardiac surgery research is steadily growing, suggesting room for further study of RHD. Simultaneously, high quality and collaborative research should be prioritized to best inform efforts to control RHD. For example, the Global Cardiovascular Clinical Trialists Forum convenes cardiovascular trialists worldwide, and the Cardiothoracic Surgical Trials Network focuses on cardiac surgical trials, albeit largely in North America and select European countries. Neither network focuses on RHD, but VHD is a common priority and future efforts may incorporate RHD.

Dozens of millions of people live with RHD yet do not receive the attention they deserve. These efforts are the first steps towards generating greater awareness about RHD, the people living with it, and the families affected by it, so that RHD can one day evolve from a disease of poverty to a disease from the past.


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