Elsevier

The Lancet

Volume 387, Issue 10025, 26 March–1 April 2016, Pages 1335-1346
The Lancet

Series
Valvular aspects of rheumatic heart disease

https://doi.org/10.1016/S0140-6736(16)00547-XGet rights and content

Summary

Acute rheumatic fever and rheumatic heart disease remain major global health problems. Although strategies for primary and secondary prevention are well established, their worldwide implementation is suboptimum. In patients with advanced valvular heart disease, mechanical approaches (both percutaneous and surgical) are well described and can, for selected patients, greatly improve outcomes; however, access to centres with experienced staff is very restricted in regions that have the highest prevalence of disease. Development of diagnostic strategies that can be locally and regionally provided and improve access to expert centres for more advanced disease are urgent and, as yet, unmet clinical needs. We outline current management strategies for valvular rheumatic heart disease on the basis of either strong evidence or expert consensus, and highlight areas needing future research and development.

Introduction

Acute rheumatic fever is a delayed auto-immune reaction to group A streptococcal infection and its long-term sequelum, rheumatic heart disease have been virtually eliminated from high-income countries where rheumatic heart disease is predominantly reported in the ageing population, a consequence of acute rheumatic fever several decades earlier, usually occurring during childhood.1, 2, 3 However, in the world's poorest populations, rheumatic heart disease remains a leading non-communicable disease of the young.4 In 2010, an estimated 34·2 million people worldwide had rheumatic heart disease, resulting in 345 110 deaths and 10·1 million disability-adjusted life-years lost per annum.4, 5 These Global Burden of Disease figures4 suggest that 0·49% of the global population have rheumatic heart disease, which could be an underestimate because of restricted global data, underdiagnoses, and few formal reporting systems.6 Acute rheumatic fever and rheumatic heart disease prevelance varies globally (figure 1).7, 8, 9 Children and adolescents aged between 5 years and 15 years are at the greatest risk of a primary episode of acute rheumatic fever, however, the peak prevalence of rheumatic heart disease is between 25–45 years of age,10 reflecting delayed case detection and the cumulative effect of recurrent episodes of acute rheumatic fever.

Section snippets

Pathogenesis

Valvular involvement, particularly, of the left-sided valves is the hallmark sign of rheumatic heart disease. It is related to endothelial damage, specifically the valves might be damaged because of their structure with a small core of connective tissue covered by two endothelial layers. Connective tissue involvement along with endothelial inflammation leads to the chronicity of the disease (figure 2).11 Histopathological studies showed that the mitral valve is universally affected, however,

Diagnosis

Diagnosis of rheumatic heart disease in individuals with an episode of acute rheumatic fever is detailed in the latest 2015 revision of the Jones criteria.18 However, most patients with symptomatic advanced rheumatic heart disease do not have a history of acute rheumatic heart disease.7, 15 In 2012, the World Heart Federation developed evidence-based echocardiographic guidelines19 to facilitate early detection in individuals both with and without history of acute rheumatic heart disease.

Morbidity and mortality

Once established, rheumatic heart disease is associated with substantial morbidity and mortality. Before the introduction of secondary penicillin prophylaxis, 20-year mortality due to acute rheumatic fever and rheumatic heart disease in the USA was as high as 30–80%, with most affected individuals dying before the age of 30 years.20, 21 Similar findings are nowadays being observed in many low-income and middle-income countries, with annual mortality of 3·0–12·5%. In some studies, the mean age

Acute rheumatic valvulitis

About 10% of patients with acute rheumatic fever develop severe acute valvulitis, mitral or aortic insufficiency, after the first episode of acute rheumatic fever as characterised by small pink vegetations (verrucae) on the leaflets, active inflammation and oedema along with focal evidence of infiltration of immune cells, and neoangiogenesis.41, 42, 43 Eradication of streptococcus and implementation of secondary prophylaxis with continuous benzathine penicillin G are the only evidence-based

Percutaneous intervention

Percutaneous mitral balloon commissurotomy (PMBV), which relies on mechanical force to split fused commissures, results in similar event-free survival and clinical events to surgery in selected patients.50 In countries with access to experienced interventional cardiological and cardiovascular surgical services, PMBV has become the reference standard for treatment of rheumatic heart disease and has been used in many clinical conditions including stable symptomatic patients, during pregnancy, and

Surgical approaches

Although PMBV has largely replaced cardiovascular surgery as the treatment for patients with dominant mitral stenosis, most patients with severe advanced rheumatic heart disease require surgical interventions. Surgical strategies have evolved in the past several years, particularly in relation to valve conserving operations. Complications (including degeneration and valve thrombosis) of all available valve substitutes used for rheumatic heart disease remain unacceptably high.55, 56, 57, 58

Tricuspid valve disease

Tricuspid valve disease usually occurs in the setting of left-sided valve involvement, most commonly severe mitral valve disease, which has led to pulmonary hypertension, right ventricular dilatation, and then functional tricuspid regurgitation.13 Less commonly, organic tricuspid valve affection can occur and lead to stenosis, regurgitation, or mixed disease. The disease process can involve all components of the tricuspid valve apparatus, however, the pattern and extent of disease is usually

Future challenges

Early detection: echocardiographic screening

Most patients are diagnosed with rheumatic heart disease after presentation to a local clinic with symptomatic heart failure without a history of acute rheumatic fever.15 At this stage, expensive cardiac surgery or percutaneous intervention is the only life-saving option. The optimum approach for early detection of rheumatic heart disease is by performing population-based screening.

Since its development the 2012 World Heart Federation diagnostic

Conclusions

Acute rheumatic fever and subsequent rheumatic heart disease are global problems of major importance that cause increased morbidity and mortality associated with substantial socioeconomic costs. Disease prevalence is affected by widespread regional variability in incidence of the disease, affected by age of the patients, mode and timing of disease presentation, and specific valvular abnormality. Unmet clinical needs for acute rheumatic fever and subsequent rheumatic heart disease include

Search strategy and selection criteria

We searched Ovid MEDLINE, Ovid Embase, Up-to-Date, Web of Science, and Scopus for reports published in English between Jan 1, 2000, and Feb 1, 2016. We did not exclude commonly referenced and highly regarded older publications. We used the search terms “rheumatic fever”, “acute rheumatic fever”, “rheumatic heart disease”, “cardiac surgery for rheumatic heart multivalvular disease”, “mitral stenosis”, “mitral regurgitation”, “aortic valve disease”, “rheumatic fever global epidemic”, “rheumatic

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