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Echocardiographic Detection of Latent Rheumatic Heart Disease

2017; Lippincott Williams & Wilkins; Volume: 136; Issue: 23 Linguagem: Inglês

10.1161/circulationaha.117.030642

1524-4539

Mohammed R. Essop, Bongani M. Mayosi,

Antimicrobial Resistance in Staphylococcus

HomeCirculationVol. 136, No. 23Echocardiographic Detection of Latent Rheumatic Heart Disease Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessEditorialPDF/EPUBEchocardiographic Detection of Latent Rheumatic Heart DiseaseA Pandora's Box? Mohammed R. Essop, MBBCh and Bongani M. Mayosi, MBChB Mohammed R. EssopMohammed R. Essop Faculty of Health Sciences, Department of Health Sciences, Division of Cardiology, University of the Witwatersrand, Johannesburg, South Africa (M.R.E). Department of Medicine, Groote Schuur Hospital, University of Cape Town, South Africa (B.M.M.). and Bongani M. MayosiBongani M. Mayosi Faculty of Health Sciences, Department of Health Sciences, Division of Cardiology, University of the Witwatersrand, Johannesburg, South Africa (M.R.E). Department of Medicine, Groote Schuur Hospital, University of Cape Town, South Africa (B.M.M.). Originally published5 Dec 2017https://doi.org/10.1161/CIRCULATIONAHA.117.030642Circulation. 2017;136:2245–2247Article, see p 2233Thomas Sydenham, an English physician, is credited with the first description of acute rheumatic fever in 1685.1 In the >300 years that have followed, notable landmarks include description of valve thickening at autopsy by Matthew Baillie in 1797, invention of the stethoscope and diagnosis of mitral valve murmurs by Rene Laennec in 1818, identification of streptococcus as a cause of pharyngitis by Theodore Billroth in 1874, recognition of the Aschoff nodule as pathognomonic of rheumatic carditis by Ludwig Aschoff in 1883, and formulation of a set of clinical criteria for the diagnosis of acute rheumatic fever by T. Duckett Jones in 1944.2 Arguably, in modern history, the unique capability of echocardiography to allow detailed insights into cardiac structure and function must rank as a milestone for cardiology in general and rheumatic heart disease (RHD) in particular. In recognition of the utility of echocardiography, in 2015, the American Heart Association included Doppler echocardiography as a major criterion in the revised Jones criteria for the diagnosis of acute rheumatic fever.3Prevalence of RHD is a litmus test of a society's state of health. The persistence of RHD in disparate regions of the world bears testimony to both economic deprivation in low-income economies and unequal access to health care in middle- and high-income regions. Before the advent of echocardiography, RHD prevalence was estimated by clinical examination of cohorts at high risk—usually children of school-going age. In 1996, Anabwani and Bonhoeffer4 were the first to sound the alarm that the prevalence of RHD using echocardiographic screening was much higher than by clinical screening alone. Several studies thereafter, using conventional or portable handheld ultrasound devices and a variety of different criteria, confirmed that the prevalence of RHD detected by echocardiographic screening was up to 10-fold higher compared with conventional methods.5 The implications of incorporating screening echocardiography for the detection of latent RHD into routine healthcare strategies are profound and need to be considered more carefully.The benefits of the detection of latent disease are dependent on the burden of subclinical disease, ease of diagnosis, sensitivity and specificity of screening tests, consequences for the individual of undiagnosed disease, potential for latent disease to serve as a reservoir for further spread, and evidence that specific treatment of undiagnosed disease would favorably alter long-term outcomes in a cost-effective manner. For these reasons, identification and treatment of latent tuberculosis infection, for example, are increasingly seen as an important component of the overall strategy for control and elimination of the disease. As attractive as screening echocardiography for latent RHD may appear to be, widespread acceptance of this technique into routine clinical practice outside the setting of a research environment has been hampered by several issues. Among others, these include the possibility that echocardiography may be too sensitive and lack specificity, resulting in an overdiagnosis of RHD, a lack of uniform echocardiographic criteria to confirm RHD, a lack of evidence pertaining to the natural history of latent RHD, and uncertainty over whether antibiotic treatment of latent RHD would favorably impact the prognosis of these patients.6In a study of 4869 Ugandan school children who underwent clinical examination and portable echocardiography, Beaton et al7 identified 130 (2.7%) with abnormal screening echocardiography. Repeat evaluation at a tertiary hospital confirmed definite, probable, or possible RHD in 72 children, giving a positive predictive value for school-based echocardiographic screening of only 56%. Of the 72 patients, 8 had definite and 17 probable RHD, both categories requiring presence of a murmur by auscultation as a diagnostic criterion. The remaining 47 patients had possible RHD defined as origin from an RHD endemic area, no cardiac murmur, and thickened valves with significant regurgitation on echocardiography. The incremental value of screening echocardiography was therefore 0.96% (47/4869) because all the other cases would have been suspected by clinical examination anyway.Grimaldi et al8 conducted an echocardiographic screening program, also in Uganda, in a similar population of 5- to 17-year-old schoolchildren. This study, which occurred during the same time period and used the same echocardiographic criteria but with some differences in the study protocol, found a 10-fold higher prevalence of definite RHD compared with the Beaton study (16.5/1000 versus 1.6/1000). The diagnostic accuracy of screening echocardiography is variable and prone to many influences, including diagnostic criteria, type of screening equipment, operator experience, and prevalence of RHD in the study population. Adoption of a uniform set of diagnostic echocardiographic criteria such as those proposed by the World Heart Foundation9 may in part alleviate some of the shortcomings of screening echocardiography for RHD, but even these criteria have been judged by some as being too complex,10 too rigid, and lacking sensitivity.11In this issue of Circulation, Beaton and colleagues12 use echocardiography to offer further insights into an immensely important but hitherto largely unexplored aspect of RHD—the manner and determinants of progression of latent RHD. The current study comprised 227 school children identified with latent RHD from a prospective Ugandan RHD registry. Using the World Heart Foundation criteria for echocardiographic diagnosis of RHD combined with the American Society of Echocardiography and American Heart Association guidelines for assessment of severity of valve dysfunction, 164 patients were classified as borderline, 42 as mild-definite RHD, and 21 as moderate/severe-definite RHD. Echocardiographic progression of disease after a mean follow-up of 2.3 years was seen in 9.8%, 26%, and 52%, and regression in 46%, 45.2%, and 9.5% of patients, respectively. Risk factors for progression included more advanced disease category, younger age, and abnormality of valve morphology. Penicillin prophylaxis was administered by physician preference in a nonrandomized fashion to 47.6%, with 84.7% compliance making it difficult to evaluate the efficacy of this therapy. Based on their findings, the authors conclude that latent RHD may have a variable trajectory; those with borderline and mild-definite disease have a significant risk of progression; patients with moderate/severe-definite disease have a poor outcome and should be treated as clinically evident cases; progression may be predicted by a young age, advanced disease, and morphological valve abnormality; and finally, the pattern of evolution of latent RHD may already become apparent within the first 1 to 2 years of establishing the diagnosis.The study by Beaton et al12 is not a randomized trial, nor is it a particularly large study, but it provides preliminary insights into the nascent stages of RHD before the occurrence of clinically advanced and largely irreversible disease. As with any novel line of investigation, the current study raises as many questions as it answers. For starters, it would have been useful to know the size of the denominator from which the cohort of latent RHD was derived, allowing one to estimate the magnitude of the problem. Furthermore, it is not patently clear whether a diagnosis of latent RHD was assigned if echocardiographic screening was positive in a patient with no history of clinical RHD or only if a careful clinical examination yielded no evidence of valvular disease. Although this distinction may appear subtle, it could sway the decision between advocating clinical examination and screening echocardiography.Progression of RHD is known to occur as a result of both ongoing inflammation and fibrosis and recurrent bouts of acute rheumatic fever. It is for the latter reason, in fact, that long-term antibiotic prophylaxis is recommended for patients with RHD. The Beaton study12 is well poised to assess the contribution of recurrent rheumatic fever to RHD progression and, even more intriguing, has the potential to identify serological markers of inflammation, heightened immunologic activity, and heart failure for predicting a more aggressive disease course. In the current study, Beaton et al12 observed regression of disease in almost half of the total group but mainly in those with borderline and mild-definite disease. A study by Zühlke et al13 of latent RHD followed for a longer period of 60 months showed regression in 52.3%, no change in 31.8%, and progression in only 15.9%. The management of such patients who regress their echocardiographic abnormalities to normal is uncertain, and indeed it is unclear whether they even should still be labeled as having RHD.The study by Beaton et al12 makes an invaluable contribution to the overall body of data on latent RHD, but it still leaves a lingering doubt whether mass screening echocardiography should be incorporated routinely into healthcare programs in developing countries. The current study sets the stage for a randomized study evaluating the benefit of intervention with antibiotic prophylaxis in patients with latent RHD. Until such a time, governments and healthcare authorities in developing countries should be urged to establish national programs based on the ASAP (application of primary and secondary prevention strategies) approach.14 When Pandora opened the lid of the box, she let out all evil and uncertainty, but she left behind hope and expectation, both in abundant need if we desire to eliminate the scourge of RHD.DisclosuresNone.FootnotesThe opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.Circulation is available at http://circ.ahajournals.org.Correspondence to: Mohammed R. Essop, MBBCh, University of the Witwatersrand, Faculty of Health Sciences, York Rd, Johannesburg, South Africa. E-mail [email protected]References1. Hajar R. Rheumatic fever and rheumatic heart disease a historical perspective.Heart Views. 2016; 17:120–126. doi: 10.4103/1995-705X.192572.CrossrefMedlineGoogle Scholar2. Jones TD. The diagnosis of rheumatic fever.JAMA. 1944; 126:481–484.CrossrefGoogle Scholar3. Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, Carapetis J, Remenyi B, Taubert KA, Bolger AF, Beerman L, Mayosi BM, Beaton A, Pandian NG, Kaplan EL; American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association.Circulation. 2015; 131:1806–1818. doi: 10.1161/CIR.0000000000000205.LinkGoogle Scholar4. Anabwani GM, Bonhoeffer P. Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography.East Afr Med J. 1996; 73:215–217.MedlineGoogle Scholar5. Rothenbühler M, O'Sullivan CJ, Stortecky S, Stefanini GG, Spitzer E, Estill J, Shrestha NR, Keiser O, Jüni P, Pilgrim T. Active surveillance for rheumatic heart disease in endemic regions: a systematic review and meta-analysis of prevalence among children and adolescents.Lancet Glob Health. 2014; 2:e717–e726. doi: 10.1016/S2214-109X(14)70310-9.CrossrefMedlineGoogle Scholar6. Essop MR, Peters F. Contemporary issues in rheumatic fever and chronic rheumatic heart disease.Circulation. 2014; 130:2181–2188. doi: 10.1161/CIRCULATIONAHA.114.009857.LinkGoogle Scholar7. Beaton A, Okello E, Lwabi P, Mondo C, McCarter R, Sable C. Echocardiography screening for rheumatic heart disease in Ugandan schoolchildren.Circulation. 2012; 125:3127–3132. doi: 10.1161/CIRCULATIONAHA.112.092312.LinkGoogle Scholar8. Grimaldi A, Ammirati E, Mirabel M, Marijon E. Challenges of using ultrasound for subclinical rheumatic heart disease screening.Int J Cardiol. 2012; 113:3081.Google Scholar9. Reményi B, Wilson N, Steer A, Ferreira B, Kado J, Kumar K, Lawrenson J, Maguire G, Marijon E, Mirabel M, Mocumbi AO, Mota C, Paar J, Saxena A, Scheel J, Stirling J, Viali S, Balekundri VI, Wheaton G, Zühlke L, Carapetis J. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease: an evidence-based guideline.Nat Rev Cardiol. 2012; 9:297–309. doi: 10.1038/nrcardio.2012.7.CrossrefMedlineGoogle Scholar10. Marijon E, Mirabel M, Celermajer DS, Jouven X. Rheumatic heart disease.Lancet. 2012; 379:953–964. doi: 10.1016/S0140-6736(11)61171-9.CrossrefMedlineGoogle Scholar11. Tani LY. Echocardiographic screening for rheumatic heart disease.Circulation. 2014; 129:1912–1913. doi: 10.1161/CIRCULATIONAHA.114.009406.LinkGoogle Scholar12. Beaton A, Aliku T, Dewyer A, Jacobs M, Jiang J, Longenecker CT, Lubega S, McCarter R, Mirabel M, Mirembe G, Namuyonga J, Okello E, Scheel A, Tenywa E, Sable C, Lwabi P. Latent rheumatic heart disease: identifying the children at highest risk of unfavorable outcome.Circulation. 2017; 136:2233–2244. doi: 10.1161/CIRCULATIONAHA.117.030642.LinkGoogle Scholar13. Zühlke L, Engel ME, Lemmer CE, van de Wall M, Nkepu S, Meiring A, Bestawros M, Mayosi BM. The natural history of latent rheumatic heart disease in a 5 year follow-up study: a prospective observational study.BMC Cardiovasc Disord. 2016; 16:46. doi: 10.1186/s12872-016-0225-3.CrossrefMedlineGoogle Scholar14. Mayosi BM. Screening for rheumatic heart disease in Eastern Nepal.JAMA Cardiol. 2016; 1:96–97. doi: 10.1001/jamacardio.2015.0303.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By de Loizaga S, Beaton A, Nascimento B, Macedo F, Spolaor B, de Pádua L, Ribeiro T, Oliveira G, Oliveira L, de Almeida L, Moura T, de Barros T, Sable C and Nunes M (2021) Diagnosing rheumatic heart disease: where are we now and what are the challenges?, Expert Review of Cardiovascular Therapy, 10.1080/14779072.2021.1970531, 19:9, (777-786), Online publication date: 2-Sep-2021. Karki P, Uranw S, Bastola S, Mahato R, Shrestha N, Sherpa K, Dhungana S, Odutayo A, Gurung K, Pandey N, Agrawal K, Shah P, Rothenbühler M, Jüni P and Pilgrim T (2021) Effectiveness of Systematic Echocardiographic Screening for Rheumatic Heart Disease in Nepalese Schoolchildren, JAMA Cardiology, 10.1001/jamacardio.2020.7050, 6:4, (420), Online publication date: 1-Apr-2021. December 5, 2017Vol 136, Issue 23 Advertisement Article InformationMetrics © 2017 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.117.030642PMID: 29203566 Originally publishedDecember 5, 2017 Keywordslatent rheumatic heart diseaseechocardiographyEditorialsPDF download Advertisement SubjectsEchocardiographyEpidemiologyPrognosis