Rheumatic Heart Disease: Important Steps to Tackle a Misunderstood Disease

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).

Learn more on ctsnet.org.