A look at Rheumatic Heart Valve Disease
By Dr Sally Chapman
Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) remain a worldwide health problem, causing premature morbidity and mortality. The highest rates of ARF and RHD are seen in developing countries, particularly Central and sub- Saharan Africa. This is largely due to poor economic status, overcrowding of homes and limited access to healthcare and antibiotics. However, there are pockets within developed countries that have high incidences of ARF/ RHD – particular Australia and New Zealand. Within these countries, the condition is predominantly seen in indigenous populations living in areas of the country where access and engagement to healthcare is challenging due to many factors including distance and geography. This also proves challenging when arranging follow up, patients will often have to travel long distances for clinic appointments, delivery of antibiotic prophylaxis and surgical assessment.
Whilst not very common in the UK, RHD is often seen in other countries in the world, including developed ones. For example, in 2015-2016 there were 2244 reported cases of acute rheumatic fever in Queensland, Western Australia, South Australia and the Northern Territories, with 95% of those cases reported in indigenous individuals (1). As of the end of 2019, there were 5385 people on RHD registers, 81% of those identified as Aboriginal or Torres Strait Islanders with 39% under 25 years of age. The burden of disease is growing, and the costs of healthcare are increasing, with the cost of managing RHD thought to be over $300 million by 2031 (2).
The most affected valve in RHD is the mitral valve, and most commonly a mixed picture of mitral stenosis and regurgitation is seen. Following the initial infection, an acute valvitis is seen which presents as mitral regurgitation. The valve then becomes scarred and fibrosed leading to a mixed lesion. Typically, in younger people, there is overriding prolapse of the anterior mitral valve leaflet. The subsequent development of thickened and restricted leaflets causes the classic ‘hockey stick’ appearance of the mitral valve (see images).
The aortic valve is less commonly affected in isolation and is often seen in patients with multi valve involvement. The predominant lesion is aortic regurgitation. The affected cusps thicken and result in rolling of the edges and ultimately a coaptation defect. Aortic stenosis alone is rarely a complication of rheumatic heart disease and other aetiologies should be considered.
The complications of RHD account for a large number of hospitalisations – heart failure, atrial fibrillation, stroke, and infective endocarditis. Heart failure and atrial fibrillation being the most commonly seen – with 19% of patients having a heart failure diagnosis at 10 years, and 13% with a diagnosis of AF.
Following diagnosis of acute rheumatic fever and/or rheumatic heart disease, patients are prescribed antibiotic prophylaxis according to the Australian Rheumatic Heart Disease guidelines. This is a monthly penicillin injection the duration of which is dependent on the severity of the valve lesion.
Although ARF and RHD are relatively rare in the United Kingdom, their global impact and the experiences of other developed nations highlight important considerations. With increasing migration to the UK, the potential to encounter RHD cases in clinical practice is likely to rise, especially among patients from regions where the disease remains prevalent. This highlights the need for healthcare systems to remain vigilant, equipped with appropriate diagnostic tools, and prepared to manage the complexities of RHD, including the ongoing care, follow-up, and prophylactic treatments that are crucial for preventing complications. By understanding the burden and management strategies employed internationally, the UK can improve readiness and ensure better outcomes for those affected by this serious but preventable condition.
 References
1. AIHW National Hospital Morbidity Database http://www.aihw.gov.au
2. Australian Acute Rheumatic Fever and Rheumatic Heart Disease Guidelines 2020 https://www.rhdaustralia.org.au/arf-rhd-guidelines