Acute Rheumatic Fever and Rheumatic Heart Disease in Resource-Limited Settings
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Downloaded from http://adc.bmj.com/ on April 28, 2017 - Published by group.bmj.com Global child health Acute rheumatic fever and rheumatic heart disease in resource-limited settings Gabriella Watson,1 Bintou Jallow,1 Kirsty Le Doare,1,2 Kuberan Pushparajah,3 Suzanne T Anderson1 ▸ Additional material is ABSTRACT working in resource-limited settings in their diag- published online only. To view Poststreptococcal complications, such as acute rheumatic nosis and management, and approaches for over- please visit the journal online fi (http://dx.doi.org/10.1136/ fever (ARF) and rheumatic heart disease (RHD), are coming some of these dif culties. archdischild-2014-307938). common in resource-limited settings, with RHD recognised as the most common cause of paediatric ACUTE RHEUMATIC FEVER 1Gambia Unit, Medical Research Council, Fajara, heart disease worldwide. Managing these conditions in Case vignette The Gambia resource-limited settings can be challenging. We review A 12-year-old Gambian girl presented to a health 2Wellcome Centre for Global the investigation and treatment options for ARF and centre complaining of lethargy, arthralgia and inter- Health Research, Imperial RHD and, most importantly, prevention methods in an mittent fever. She was diagnosed with clinical College, London, UK 3Department of Congenital African setting. malaria and treated accordingly. Heart Disease, Evelina London Four months later she presented to clinic with Children’s Hospital, Guy’s&St similar symptoms. On examination she looked Thomas’ NHS Foundation INTRODUCTION acutely unwell with a soft systolic murmur in the Trust, London, UK Infections caused by Group A streptococcus (GAS) mitral area. Blood tests showed leukocytosis and a fi Correspondence to were identi ed as the ninth leading cause of global raised erythrocyte sedimentation rate (ESR) of Dr Gabriella Watson, Gambia mortality from an individual pathogen in the 2004 130 mm/h. Chest radiograph (CXR) showed car- Unit, Medical Research World Health Report.1 The WHO reported 18.1 diomegaly (figure 1A). ECG showed prolonged PR Council, PO Box 273 Fajara, million people living with serious GAS disease interval, and ECHO demonstrated a moderately The Gambia; [email protected] (acute rheumatic fever (ARF), rheumatic heart dilated left ventricle and left atrium with moderate disease (RHD), RHD-related stroke or infective mitral regurgitation (see figure 1B and online sup- Received 26 December 2014 endocarditis and invasive GAS diseases (iGAS)) and plementary video). A throat swab grew GAS. She Revised 2 February 2015 5 170 000 deaths in 2005. The majority of deaths was diagnosed with ARF and admitted for inpatient Accepted 3 February 2015 were attributable to RHD complications, iGAS or treatment; bed rest with mobilisation as symptoms acute poststreptococcal glomerular nephritis allowed, intramuscular benzathine penicillin (APSGN). Close living conditions, overcrowding 900 mg, aspirin 50 mg/kg/day in five divided doses, and poor hygiene promote transmission, while lisinopril 10 mg once daily and frusemide 40 mg poor public health infrastructure prevents early rec- twice daily. Her symptoms improved, and she was ognition, prompt treatment and appropriate pre- discharged 7 days later on monthly intramuscular vention measures.2 While ARF is debilitating, it is benzathine penicillin, regular frusemide, lisinopril the long-term sequelae of RHD that are the most and 3 months of aspirin. Outpatient follow-up was devastating; young children and adolescents pre- arranged 2 weeks later for repeat ESR and to senting with limited exercise capacity and young monitor progress. women presenting in pregnancy or post childbirth She missed her initial follow-up appointment in congestive cardiac failure (CCF). Data from the due to financial constraints, but presented paediatric cardiac clinic at the Medical Research 2 months later complaining of worsening exercise Council Gambia Unit have demonstrated a median tolerance since her medications finished. On exam- age of presentation with symptomatic RHD of ination she had a raised jugular venous pressure, 11 years (work in preparation, Watson et al). A sys- pedal oedema, hepatomegaly of 6 cm and bilateral tematic review by Carapetis et al3 of GAS diseases basal crackles on chest auscultation. Repeat ECHO estimated 1.88 million existing cases of ARF glo- revealed worsening mitral regurgitation. She was bally and 180 000 new cases annually. diagnosed with RHD with CCF and admitted for RHD is the most common cause of paediatric treatment with intravenous frusemide 1 mg/kg heart disease worldwide.4 The estimated prevalence twice a day, lisinopril 10 mg once daily, intramuscu- among 5 year olds to 14 year olds in Sub-Saharan lar benzathine penicillin 900 mg and restarted on Africa is 5.7/1000 compared with 0.3/1000 in aspirin 50 mg/kg/day in five dived doses. Her symp- developed countries.3 More recent echocardio- toms gradually improved, and she was discharged graphic (ECHO) screening programmes in to home 6 days later on her previous medications Mozambique and Uganda have shown prevalence and the importance of regular outpatient follow-up rates of RHD in schoolchildren of 30.4/1000 and emphasised. 14.8/1000, respectively.56With RHD accounting for 5.9 million disability-adjusted life-years lost in Diagnosing acute rheumatic fever: are the Jones fi To cite: Watson G, 2002, this poses a signi cant medical and socio- criteria appropriate for high-prevalence Jallow B, Le Doare K, et al. economic burden on populations.1 In this article, settings? Arch Dis Child we review the cardiac complications of iGAS ARF is a short-lived, multisystem, autoimmune – 2015;100:370 375. disease, the challenges faced by the clinician disease that progresses to RHD in up to 80% of 370 Watson G, et al. Arch Dis Child 2015;100:370–375. doi:10.1136/archdischild-2014-307938 Downloaded from http://adc.bmj.com/ on April 28, 2017 - Published by group.bmj.com Global child health What investigations are required to diagnose ARF? All patients should be admitted ensuring appropriate investiga- tions, management and education. Box 1 outlines the investiga- tions recommended—these may have to be adapted depending on diagnostic facilities available. In particular, absence of cardi- ologists, appropriate ultrasound equipment or staff skilled in completing and interpreting findings may make cardiac ECHO unfeasible. How do you manage a patient with ARF? Patients with carditis and heart failure should be put on bed rest. The initial use of salicylates, corticosteroids or non- steroidal anti-inflammatories should be avoided until clinical diagnosis is established as diagnostic symptoms can be masked.8 Table 2 illustrates treatment options summarised from the WHO and Australian guidelines for the management of ARF. RHEUMATIC HEART DISEASE Composite case vignette A 15-year-old boy presented to our clinic with fever, cough, leg swelling and poor exercise tolerance for 4 months. On examin- ation he was wasted, weight of 35 kg, short of breath at rest, had a soft systolic murmur and an irregular heart rate. He was admitted for investigation and treatment. CXR showed massive cardiomegaly and pulmonary plethora. ECHO demonstrated significantly thickened and regurgitant aortic and mitral valves (MV) resulting in a hugely dilated left atrium and ventricle. The tricuspid valve was similarly affected. Ventricular function was preserved, with an ejection fraction of 70%. He had an ESR of 50 mm/h without lymphocytosis and three negative blood cul- tures. Throat swab showed no growth. He was diagnosed with multivalvular RHD and CCF and managed with intravenous frusemide, lisinopril, penicillin prophylaxis and discharged 6 days later. In the following year, he had eight further admissions with decompensated heart failure. Given the limited medical and sur- Figure 1 (A) A plain chest radiograph demonstrating an enlarged gical resources for patients with RHD in The Gambia, definitive cardiothoracic ratio due to cardiomegaly. The carina is also splayed due surgical treatment (mitral and tricuspid annuloplasty and mech- to the marked enlargement of the left atrium (LA). There is coexisting anical aortic valve (AV) replacement) was performed overseas, evidence of pulmonary congestion. (B) A parasternal long-axis funded by a charitable organisation. He made a rapid post- echocardiographic view demonstrating a markedly dilated LA. The operative recovery and is currently managed on intramuscular mitral valve (MV) leaflets appear thickened and have restricted Benzathine Penicillin, lisinopril, bisoprolol and warfarin adjusted movement with a typical ‘hockey stick’ appearance of the anterior MV leaflet. This view is shown as a moving image in online supplementary according to monthly international normalised ratio (INR) mea- fi video. LV, left ventricle. surements. He has good exercise tolerance and signi cantly improved nutritional status. cases. There is no specific laboratory test for ARF and diag- How do you diagnose RHD in low-resource settings? nosis remains a clinical one, based on major and minor cri- ECHO is the most sensitive and specific diagnostic tool for teria first outlined by Jones in 1944. Changes have been RHD,9 and standardised ECHO criteria for diagnosing asymp- made to increase specificity at the expense of sensitivity, tomatic RHD exist.10 The most commonly affected valves are primarily for resource-rich countries. However, in areas the MV and AV. Morphological features of MV disease in RHD where the risk of underdiagnosis may outweigh