The following are key points to remember from this review on rheumatic heart disease (RHD):

Epidemiology:

1. The global burden of RHD continues to be significant. In 2017, there were an estimated 38-40.8 million cases of RHD globally, with the highest prevalence in Oceania, South Asia, and sub-Saharan Africa.
2. Prevalence ranged from 3.4 cases/100,000 in non-endemic regions, to >1,000/100,000 cases in endemic areas.
3. Data on RHD related morbidity and mortality are less robust, but the estimate is at least 260,000-300,000 deaths per year.
4. A global registry of 3,300 RHD cases from 14 lower- to mid-income countries reveals that most patients with RHD are young (median age 28 years), female (66%), with moderate to severe multivalvular disease (64%) complicated by congestive heart failure (33%), pulmonary hypertension (29%), atrial fibrillation (22%), and stroke (7%).
5. The World Heart Federation (WHF) has set forth an aim to reduce the burden of RHD by 25% in 2025.

RHD diagnosis:

1. During acute rheumatic fever (ARF), rheumatic carditis can manifest as pericarditis or valvulitis. Rheumatic carditis will frequently progress to RHD (up to 70% in certain studies), although the initial ARF will have often been missed.
2. The mitral or both mitral and aortic valves are most commonly affected. Isolated aortic valve, or right-sided valve involvement is rare. Acute mitral valvulitis can result in anterior leaflet prolapse, annular dilation, chordal elongation, and varying degrees of mitral regurgitation. Over time, chronic inflammation results in commissural fusion with involvement of the mitral valve apparatus, resulting in mitral stenosis.
3. The WHF has well-defined minimal echocardiography criteria for the diagnosis of RHD, while the American Society of Echocardiography/American College of Cardiology/American Heart Association guidelines further outline well-recognized criteria for quantification of degree of regurgitation or stenosis. Research is being done on more simplified criteria that could be obtained on cheaper hand-held machines by less skilled/trained technicians.
4. Echocardiographic assessment of the mitral valve apparatus should include leaflet mobility, valve thickening, subvalvular thickening, valvular calcification, commissural morphology, and leaflet displacement—all of which are needed to determine the likelihood of successful intervention with balloon mitral valvuloplasty.

Screening:

1. Improved identification of ARF and prompt initiation of treatment could reduce progression to RHD and is, therefore, of great interest. However, fever is nonspecific and up to one third of patients with ARF report no history of sore throat.
2. Auscultation is neither sensitive nor specific for detection of RHD. However, limited prevalence data have made it difficult to support echocardiography-based screening.
3. The two most suitable populations for echo-based screening are school-aged children, as they would still benefit from secondary prophylaxis, and pregnant women, given the potential consequences for both mother and baby.
4. Echo-based screening has led to creation of multiple registries, which track the prevalence of RHD and its natural progression. These have established the need for long-term antibiotic treatment.
5. The role of anti-streptolysin O titers in determining treatment or monitoring efficacy of prophylaxis in subclinical RHD remains unclear.
6. The role of echocardiographic screening as a public health strategy for global reduction of the burden of RHD, its related morbidity and mortality, and estimates of number needed to treat are still unknown.

Management:

1. The key to ARF/RHD management is secondary prevention with continuous antibiotic prophylaxis to prevent recurrent infection with Group A streptococcus. Benzathine penicillin G dosed every 3-4 weeks is superior to oral penicillin.
2. Data on appropriate duration of treatment are based mostly on expert opinion and vary among different countries. Considerations include ARF presentation (age, time since last ARF, ± rheumatic carditis), and presence and severity of chronic RHD. Typical treatment durations are 5-10 years, or until age 21 (whichever is longer). For severe chronic RHD, treatment can be life-long, even after surgical intervention.
3. For patients under age 35 years without a documented history of ARF, treatment durations are a minimum of 5 years or until age 40 (whichever is longer). Life-long prophylaxis is recommended following valve surgery.
4. While typical guidelines for severe valvular heart disease stress surgical and catheter-based interventions, the majority of cases occur in regions of the world where these options may not be available. Typical agents such as diuretics, afterload reducers, and beta-blockers are recommended for symptomatic relief of heart failure.
5. For atrial fibrillation or flutter, anticoagulation with oral vitamin K antagonists or direct oral anticoagulants is still recommended. However, the INVICTUS-VKA study is currently evaluating noninferiority of rivaroxaban to warfarin.
6. For isolated mitral stenosis in symptomatic patients with favorable valve anatomy, balloon mitral valvuloplasty is generally preferred given the lower cost and rapid recovery time. While complications (such as tamponade or valve leaflet rupture) are rare (2-5%), on-site surgical back-up is typically still required. Long-term benefit after balloon mitral valvuloplasty is seen in about 75% of patients.
7. While surgical mitral valve repair by experienced surgeons is feasible in >75% of cases, the most important consideration in RHD-endemic regions is limiting the risk of a redo operation. This makes valve replacement the more common practice, especially for double-valve surgery (with a subsequent need for lifetime anticoagulation).
8. Access to surgeons remains the most important problem in RHD endemic areas, with three cardiothoracic surgeons per 1 million inhabitants in North Africa and one cardiothoracic surgeon per 3.3 million people in Sub-Saharan Africa. International declarations to improve access to surgery in endemic areas through global alliances and structured training of more cardiac surgeons will be essential.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Pericardial Disease, Prevention, Valvular Heart Disease, Anticoagulation Management and Atrial Fibrillation, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Acute Heart Failure, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound, Mitral Regurgitation

Keywords: Anticoagulants, Anti-Bacterial Agents, Antibiotic Prophylaxis, Atrial Fibrillation, Balloon Valvuloplasty, Cardiac Surgical Procedures, Constriction, Pathologic, Dilatation, Diuretics, Diagnostic Imaging, Echocardiography, Heart Defects, Congenital, Heart Failure, Heart Valve Diseases, Mitral Valve Insufficiency, Mitral Valve Stenosis, Myocarditis, Penicillin G Benzathine, Pericarditis, Pregnancy, Rheumatic Fever, Rheumatic Heart Disease, Secondary Prevention, Stroke, Warfarin

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