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Acute rheumatic fever results from an autoimmune response to infection with a group A streptococcus resulting in multi-organ involvement., Except for carditis, none of its manifestations lead to permanent damage.Rheumatic fever and its consequences (rheumatic heart disease) remain one of the devastating diseases affecting the Pacific people wherever they are around the world.1-9 There are nearly 16 million people worldwide who suffer from rheumatic heart diseases (RHD). More than 200,000 deaths each year are due to the disease and its sequealae.10 The vast majority of the burden is borne by the developing countries.Acute rheumatic fever (ARF) and RHD are becoming less common in developed countries as living conditions, hygiene, access to medical care, nutrition and socioeconomic standards improve. Unfortunately within some of the developed countries like USA, Australia, New Zealand and Hawaii, the presence of ARF and RHD are still quite prevalent amongst its indigenous and migrant populations particularly those with origins in the Pacific. With ARF and RHD so prevalent amongst the Pacific people living in the islands or in other countries, this suggests a genetic link or predisposition. Unfortunately the genetic link has not yet been established.11Data collection to assess the burden of ARF and RHD in any country is always a challenge because of the history of the disease and the health infrastructure. This is especially true in the Pacific and Samoa. The Jones Diagnostic Criteria for ARF is difficult to apply to rheumatic fever cases in Samoa because people present late, with recurrent symptoms. In our experience, many of our people present at the stage of established RHD rather than at the sore throat and ARF stage.Epidemiology of acute rheumatic fever (ARF) and rheumatic heart disease (RHD)In Samoa the reported incidence of ARF was 16.1 per 100,000 in 1985 and the prevalence of RHD was 2.3 per 1000 in 1986.3 In 2000 the incidence of ARF was 35 per 100,0001 from those who were referred from all the clinics and hospitals with the diagnosis of ARF. In Hawaii the incidence of ARF in Samoans was 206 per 100,000 in 1984.8 The incidence of RHD in Samoa was 66 per 100,000 in 2005 and 68 per 100,000 in 2007 using the Echocardiogram Register.1The Echocardiogram Register recorded everyone who had had an echo study including all those who were diagnosed with ARF, those who were referred because of murmurs, and those who were referred for cardiac assessment for other reasons like heart failure, ischaemic heart disease, and congenital heart diseases. The School Auscultation Survey for Heart Murmurs by Steers and Adams in 199612 implied a RHD prevalence of 77.8 per 1000 in schools. Unfortunately there was no echocardiogram used.In 2000 and 2001, our Rheumatic Fever Team under NZAID funding performed an auscultation study for RHD in schools on 2828 kids from age 5 to age 13. Those who presented with murmurs were referred for echocardiography. The prevalence of heart murmurs was 18 per 1000, and after echocardiography the prevalence of new RHD was 3 per 1000.13,14 ARF and RHD are common in other Pacific countries.Tonga had RHD prevalence in school children of 33.2 per 1000 in 2008, and the prevalence increased with age peaking at 42.6 per 1000 in children 10-12 years old.4 This survey of 5053 of primary school children was initially conducted with auscultation and those with heart murmurs progressed to echocardiography. Auscultation will miss about 20-30% of RHD15 and therefore the actual RHD prevalence may be higher than reported if all the children were scanned.Fiji had an ARF incidence of 15.2 per 100,000 from 2005-2007 in children age 5-15 years.5 The prevalence of RHD in school children age 5-15 years old was 4.1 per 1000 for definite RHD and 8.4 per 1000 for definite and probable RHD.6 This survey of 3462 of primary school children was initially conducted with auscultation and those with heart murmurs progressed to echocardiography.During the 1950s and 1960s there were 20-30 per 100,000 cases of ARF in New Zealand. The prevalence of ARF amongst Pacific people in New Zealand was high. The incidence of ARF in New Zealand had declined to 2.8 per 100,000 from 1995-2000, 1.9% per 100,000 in 2005, and 2.5 per 100,000 in 2006.7,16Pacific people comprise about 30% of cases where ethnicity was recorded and M ori was 62%. The highest rate of ARF was in the 10-14 year old age group amongst the Pacific people (16.1 per 100,000) residing in Auckland. Some of the reported RHD prevalence was 18.6 per 1000 in Cook Island, 8.0 per 1000 in French Polynesia, and 10 per 1000 in New Caledonia.2,3,17As many people in Samoa have relatives in New Zealand there is a high incidence of migration between Samoa and New Zealand every year. This may affect the epidemiology of rheumatic fever and RHD in New Zealand and may have huge implications in the management and the control of this disease.Rheumatic Fever Programme in SamoaThe Rheumatic Fever Programme in Samoa started in the mid-1970s and was re-established in 1984. In 2000 the NZAID organisation assisted with 2-3 year funding which enabled a school-based rheumatic fever prevention pilot programme. In 2006 funding was secured from Vodafone Foundation for establishing a RHD Project from 2007 to 2009 mainly to employ dedicated staff to oversee the Rheumatic Fever Programme. The funding was administered through the World Heart Federation with technical advice from Menzies Research Centre. The Samoan Cabinet and the Ministry of Health appointed Professor S Viali (Dean of the Oceania University of Medicine) as director, RN Puleiala Saena as the nurse, and Vailogoua Futi as the secretary and field assistant.The Project was coordinated from the main National Hospital Tupua Tamasese Meaole (TTM) in the Rheumatic Fever Centre (RFC). People with RF and RHD were seen in the TTM Paediatric Clinic (if <13 years), TTM Medical Clinic (if 226513 years), Tuasivi District Hospital (in Savaii), Safotu District Hospital (Savaii), and the RFC. Echocardiograms were mainly performed by Dr S Viali in adults and kids with RF and RHD in the RFC, and in the Paediatric Clinic by Dr F Fatupaito, and sometimes by Dr L Fiu in the Medical Clinic.The information on people diagnosed with RF or RHD from all centres was regularly collected by our secretary to enter into our database. Intramuscular (IM) penicillin was delivered mainly through the RFC by RN Pule, Paediatric Clinic, Tuasivi District Hospital and Safotu District Hospital. Medical, cardiac and echocardiogram follow-up were mainly done in the RFC, TTM Paediatric Clinic and TTM Medical Clinic.There has been strong focus on public awareness and education regarding ARF and RHD.Multiple workshops on rheumatic fever were carried out for the medical and nursing staff in 2007 and 2008 using local and overseas experts. Mobile clinics and echocardiograms were done by Dr S Viali in the outer villages of Upolu and Savaii, and he was occasionally accompanied by an overseas cardiologist. Several rheumatic fever prevention programmes were frequently aired on national television (TV1 and TV3) between 2007-2009 and many articles on rheumatic fever prevention have been published in several popular local newspapers.NZAID also supported a rheumatic fever primary prevention programme for 2006 and 2007 under the Ministry of Health. This focused on health education and health promotion in rheumatic fever.Rheumatic Fever RegisterRegister-based rheumatic fever programmes have been successful in countries like New Zealand, Australia, India, Cuba and Egypt.7,18-21 To ensure efficient delivery of services and prophylaxis, and to monitor service delivery, it is crucial that the Register be accurate and up-to-date with people with ARF (known and past) and RHD. It is also a very important epidemiological tool. The most important outcome is the improvement of the status of rheumatic fever and rheumatic heart disease control.The Rheumatic Fever Programme in Samoa kept a manual register since 1984 which was described earlier.1 This manual register was well kept from 1984 to 2002 but unfortunately this register was lost. The rheumatic fever work and clinics continued with less coordination between the various centres in both islands looking after rheumatic fever patients. During this time the patients presenting with ARF and RHD were recorded in the hospital health information data base.The recent RHD project provided an electronic rheumatic fever register and all the rheumatic patients have been recorded in this database. The RHD project officially started in the beginning of 2007 hence a lot of the old rheumatic fever and RHD patients that were not known were entered, resulting in large numbers entered in 2007 into the electronic register. There were 133 people recorded with RHD in 1974,22 and 354 recorded with RHD in 1986 3, and about 708 were recorded with RHD in the current register in 2009.\r\n Table 1. Rheumatic Fever Register 2003-2009 \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n Disease\r\n \r\n \r\n \r\n 2003\r\n \r\n \r\n \r\n 2004\r\n \r\n \r\n \r\n 2005\r\n \r\n \r\n \r\n 2006\r\n \r\n \r\n \r\n 2007\r\n \r\n \r\n \r\n 2008\r\n \r\n \r\n \r\n 2009\r\n \r\n \r\n \r\n \r\n \r\n ARF\r\n New RHD\r\n Known RHD & ARF\r\n \r\n \r\n \r\n 4\r\n 121\r\n \r\n \r\n \r\n 3\r\n 87\r\n \r\n \r\n \r\n 7\r\n 46\r\n \r\n \r\n \r\n 3\r\n 43\r\n \r\n \r\n \r\n 23\r\n 63\r\n 188\r\n \r\n \r\n \r\n 13\r\n 55\r\n 20\r\n \r\n \r\n \r\n 17\r\n 49\r\n 11\r\n \r\n \r\n \r\n \r\n \r\n Total\r\n \r\n \r\n \r\n 125\r\n \r\n \r\n \r\n 90\r\n \r\n \r\n \r\n 53\r\n \r\n \r\n \r\n 46\r\n \r\n \r\n \r\n 273\r\n \r\n \r\n \r\n 88\r\n \r\n \r\n \r\n 77\r\n \r\n \r\n \r\n \r\n \r\n ARF=acute rheumatic fever; RHD=rheumatic heart disease. Those with ARF either presented or referred to the clinics from GPs or hospitals and some were admitted to the hospitals with acute symptoms of rheumatic fever. The new RHD were those who were referred because of a heart murmur and were found to have RHD, or were admitted for something else and were found to have RHD. Patients with known diagnosis of ARF or RHD were recorded in the year they were identified. The register recorded 314 people from 2003-2006 and 438 people from 2007-2009 (Table 1), 42% were males, and 6.8% have died. The incidence of ARF in Samoa has steadily reduced from 35 per 100,000 in 2000,1 to 30 per 100,000 in 2005, 12.8 per 100,000 in 2007, 7.3 per 100,000 in 2008 and 9.5 per 100,000 in 2009 (Figure 1). \r\n Figure 1. Incidence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) in Samoa For those who presented with ARF, about half had already developed carditis. This may mean that some of these presentations were of recurrent rheumatic fever instead of their first attack. About 1-3% of those with the first ARF attack presented with carditis and with subsequent attacks 25-75% would develop carditis.23,24 The most common age group that presented with ARF was 10-14 years old (57.7%), followed by 15-19 years old (19.2%) and 5-9 years old (17.2%), and none were recorded below the age of 4. The incidence of RHD per 100,000 has steadily decreased from 66 in 2005, to 40.2 in 2007, 34 in 2008, and to 31.8 in 2009 (Figure 1). The most common age group with RHD was 10-14 years old (35.7%), followed by 15-19 years old (26.8%), followed by 5-9 years old (12.1%) and 20-24 years old (10.2%), with only one RHD case recorded below the age of 4. The villages with the most ARF and RHD were in the Upolu urban areas in Apia (Vaitele, Vaiusu, Siusega, Lotopa, Sinamoga, Faatoia, Vaivase) with low numbers in Savaii. The Register has also shown the many undiagnosed RHD presenting in other ways and without ARF symptoms. It is always assumed that the presence of RHD meant that ARF must have occurred at some point in time, whether it was symptomatic or not. The acquiring of symptoms of ARF depended on many factors. The manifestations of major and minor Jones criteria vary in different countries which led to the original Jones criteria changing to the current form. From the current register, for every patient that had been diagnosed with ARF symptoms, there would be another 3-4 with undiagnosed RHD. Penicillin injections compliance Compliance to IM penicillin from 2001-2006 was estimated to be <50% according to the staff working with rheumatic patients, with many patients declining follow-up visits. Compliance was assessed by the numbers of injections per year per patient. With more focus on rheumatic fever by the Ministry of Health, National Health Services and the new Rheumatic Project, the compliance to IM penicillin improved to 74-84% in the four injection centres (RFC TTM, Paediatric Clinic TTM, Tuasivi District Hospital, Safotu District Hospital) (Figure 2). \r\n Figure 2. Compliance to IM penicillin in the four injection centres Those who did not turn up for their injections were phoned by the nurse. Previously the rheumatic fever nurse would take the penicillin injections to the homes of the rheumatic fever patients when they did not turn up. Due to better access to hospitals and improved transportation infrastructure around the island, it is now considerably easier to get the IM penicillin injections. The key to better compliance was enthusiastic, dedicated staff in the Rheumatic Fever Programme and the reminder phone call to remind the patients of the injections. In Savaii the reminder messages were relayed by health staff in the various villages. RHD cardiac surgery Cardiac surgery is very expensive, and small island economies find it difficult to afford these prices.25,26 Samoa has sent the majority of its cardiac patients to New Zealand for surgery since the 1970s. Prior to 1996 less than 20 RHD operations per year were performed in New Zealand on our patients. From 1997 to 2009 between 20 and 28 RHD operations per year (NZ$28,000-NZ$44,000 per valve operation) were performed (Figure 3). The total cost ranged from NZ$560,000 to NZ$1,300,000 per year (>ST$1.9 million tala per year). \r\n Figure 3. Cardiac surgery 1992-2009 In 2003 there were 184 referrals to New Zealand for treatment including 61 (33.3%) referrals for cardiac surgery. For cardiac surgery in 2003, 41% of these were for RHD, 26% for ischaemic heart disease (IHD), 20% for congenital heart disease (CHD) with 15% for others. The 25 RHD surgeries done in 2003 cost about NZ$1.1 million dollars (>ST$1.65 million tala) 2014a significant dent in the Samoan health budget. In 2008 there were 227 referrals to New Zealand including 62 referrals for cardiac surgery 201434% were for RHD, 40% for IHD, 21% for CHD, and 5% for others. In 2009 there were 288 referrals including 84 referrals for cardiac surgery 201431% were for RHD, 43% for IHD, 18% for congenital heart disease, and 8% for others. Though RHD surgery has declined there is a noticeable rise in IHD surgeries as there is a rise in non-communicable and lifestyle diseases in Samoa and the Pacific. Samoa 2019s overseas treatment scheme funded by the Government of Samoa, increases annually and has peaked at ST$10 million tala in 2009. In addition to the above budget, New Zealand AID annually provides about NZ$500,000. There have been ongoing discussions regarding reducing prices for cardiac surgery for Samoa but the recent recession has deferred these discussions. The alternative was to perform these surgeries in Samoa by visiting cardiac teams. In 2007 the cardiac medical mission started with 10 RHD cases operated in Samoa with no fatalities. In 2008, 14 RHD cases were done in Samoa. Unfortunately 50% developed significant pericardial effusion and 3 patients died in Samoa from cardiac tamponade. There are more cardiac operations planned for 2011. There is no question that it is economical to perform cardiac surgery (valve surgery) in Samoa.. Cardiac surgery in New Zealand is very expensive and Samoa 2019s health budget would not be able to sustain the number of surgeries needed. The key to continued success of the good-will cardiac medical mission would be the selection of low risk cases to do safely in Samoa, because the complex infrastructure needed for post-surgical care is not available. The enthusiasm and commitment of the medical team and everyone involved from New Zealand and Samoa is commendable. Echocardiogram screening for RHD in schools People in Samoa usually do not present with acute rheumatic fever symptoms, similar to Fiji,5but present with RHD symptoms, most commonly heart failure. RHD is the dreaded complication of ARF that may influence the prognosis. This has prompted the screening programme in schools. The main purpose is to quickly identify as many RHD as possible, begin early intervention with penicillin and prevent the progression of the RHD and recurrence of ARF. Most of the early survey in schools for RHD was with auscultation. Many recent RHD surveys have been done with auscultation before progressing to echocardiogram4,6 but there were significant numbers of RHD that were missed. Some surveys were done with echocardiogram which had good identification rates of RHD in asymptomatic people.27 The skill of auscultation is cheaper and more accessible than echocardiogram machines, but the identification rate for RHD using auscultation is far inferior to echocardiography.15 For auscultation, years of experience are required before being able to diagnose with certainty. Echocardiography also requires time to acquire the necessary skills to be able to diagnose adequately.. There is no doubt that echocardiography is superior to auscultation in diagnosing RHD, other heart diseases and normal flow murmurs. Though the role of echocardiography in screening is controversial at present, its specificity and application to the natural history and time course of rheumatic fever is currently being revised. On the other hand our patients in Samoa mostly present to health facilities in the advanced heart disease stage and hence screening with echocardiography in schools will certainly pick up those with significant RHD early. There is a current echocardiogram screening programme for RHD in Primary Schools in Samoa for ages 5-13 years old (year 1 to year 8) which will be completed in another 12 months. To date, 3200 kids have been screened from public and private schools in the urban region of Upolu, and the prevalence rates of RHD were similar to Tonga.4,28 There were more RHD in the public schools compared to private schools, and many children with RHD were living in the urban areas of Upolu. The Rheumatic Fever Register suggested that this screening programme should also involve secondary schools (age 14-19 years). Those that were picked up with RHD received penicillin prophylaxis, since penicillin is very effective in preventing progression of RHD.29 The definition of RHD is based on the new diagnostic criteria (morphological changes and Doppler findings).30-32 Summary The incidence of rheumatic fever and rheumatic heart disease in Samoa has decreased. Though it is still 5 times higher than the incidence in New Zealand, it is similar to the situation in Tonga and Fiji. Disease patterns have changed from the early 1900s from infectious disease to non-communicable diseases. Living standards and socioeconomic status have certainly improved which could explain the decline in the incidence of ARF and RHD. None of the ARF presented under the age of 4. About 5% of ARF could occur below the age of 5 years 33. The current rheumatic fever programme is making a difference to the follow-up of patients with ARF and RHD, and the compliance to the IM penicillin prophylaxis programme. The compliance has certainly improved with simple and inexpensive measures. The prevention programmes have also been strengthened. These measures may have also contributed to the decreasing incidence of this disease. The costs of RHD are very high for a small country like Samoa and it is not sustainable in the long term, hence the prevention of the development of ARF and early identification of RHD are some of the important strategies to reduce the burden and suffering of RHD. It is also unfortunate that many of our patients do not present with acute symptoms but with established RHD, hence screening for RHD in schools may be the most effective way of reducing the overall burden of RHD. By treating the many undiagnosed RHD with penicillin, this will prevent the progression of cardiac valve damage thus reducing suffering to the patients and the cost for cardiac surgery.\r\n \r\n \r\n \r\n \r\n

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Satupaitea Viali, Specialist Physician & Cardiologist, Director of Rheumatic Fever Project, Medical Specialist Clinic & National Health Services; Puleiala Saena, Rheumatic Fever Nurse, National Health Services; Vailogoua Futi, Secretary, National Health Services; Apia, Samoa

Acknowledgements

Correspondence

Dr Satupaitea Viali (MPH, FRACP, FCSANZ), Medical Specialist Clinic, PO Box 2122, Apia, Samoa

Correspondence Email

satu.viali@gmail.com

Competing Interests

None known

'- Viali S. Rheumatic Fever and Rheumatic Heart Disease in Samoa. Pacific Health Dialogue 2006;13(2):31-38.-- Steer A, Colquhuon S, Viali S, et al. Control of Rheumatic Heart Disease in the Pacific Region. Pacific Health Dialogue 2006:13(2):49-55.-- Neutze JM. Rheumatic fever and rheumatic heart disease in the Western Pacific region. NZ Med J 1988:101:404-406.-- Carapedis J, Hardy M, Fakakovikaetau T, et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan school children. Nature Review Cardiology 2008;(5),411-417.-- Steers AC, Kado J, Jenny AW, et al. Acute rheumatic fever and rheumatic heart disease in Fiji: prospective surveillance, 2005-2007. MJA 2009;190(3):133-135.-- Steers AC, Kado J, Wilson J, et al. High prevalence of rheumatic heart disease by clinical and echocardiographic screening among children in Fiji. J Heart Valve Disease 2009;18(3):336.-- Thornley C, McNicholas A, Baker M, Lennon D. Rheumatic Fever Registers in New Zealand. New Zealand Public Health Report 2001:8(6):41-44.-- Chun RT, Reddy V, Rhoads GG. Occurrence and prevention of rheumatic fever among ethnic groups of Hawaii. Am J Dis Child 1984;138(5):476-478.-- Miyake CY, Gauvreau K, Tani LY, et al. Characteristics of children discharged from hospitals in the United States in 2000 with the diagnosis of Acute Rheumatic Fever. Paediatrics 2007;120;503-508.-- Carapedis J, McDonald M, Wilson N. Acute rheumatic fever. Lancet 2005;366:155-168.-- Bryant PA, Robins-Browne R, Carapedis JR, Curtis N. Some of the people, some of the time: Susceptibility to Acute Rheumatic Fever. Circulation 2009;119;742-753.-- Steer AC, Adams J, Carlin J, et al. Rheumatic heart disease in school children in Samoa. Archives of Diseases in Childhood 1999;81(4):373.-- Viali S. Echocardiogram findings on children with murmurs picked up by the School-based Rheumatic Fever Prevention program. A preliminary report to the Ministry of Health, Samoa. 2001.-- McCleland V. Final Report - Process Evaluation of school based rheumatic fever prevention pilot programme. Ministry of Health, Samoa. 2002.-- Abernethy M, Bass N, et al. Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever. Aust NZ J Med 1994;24:530-35.-- ESR. Notifiable and other diseases in New Zealand: Annual report 2006. Wellington: ESR; 2007-- Rheumatic fever and rheumatic heart disease. WHO 1988.-- Strasser T, Dondog N, El Kholy A, et al. The community control of rheumatic fever and rheumatic heart disease: a report of a WHO international cooperative project. Bull World Health Organ 1981;59:285-294.-- Kumar R, Raizada A, Aggarwal AK, et al. A community-based rheumatic fever / rheumatic heart disease cohort: twelve-year experience. Indian Heart Journal 2002:54:54-58.-- World Health Organization. Rheumatic fever and rheumatic heart disease: report of a WHO expert consultation. Geneva: WHO; 2004.-- Noonan S, Edmond K, Krause V, et al. The top end rheumatic heart disease control program I. Report on progress. NT Dis Control Bull 2001;8:15-18.-- Smith WM. An appraisal of rheumatic valvular heart disease in Western Samoa. Aust New Zealand J Med 1979;9:560-65.-- Siegel AC, Johnson EE, Stollerman GH. Controlled studies of streptococcal pharyngitis in a paediatric population, 1: factors related to the attack rate of rheumatic fever. NEJM 1961;265;559-565.-- Manyemba J, Mayosi BM. Penicillin for secondary prevention of rheumatic fever. Cochrane Database Syst Rev 2002;(3):CD002227.-- Viali S. Cardiology and Cardio-surgical Report to the Ministry of Health, Samoa. 2003.-- Viali S. Clinical Medical Audit Presentation. Samoa Medical Association Medical Seminar, Apia, Samoa; 2004.-- Marijion E, Ou P, Celermajer DS, Ferreira B, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. NEJM 2007;357:470-476.-- Viali S. Report to the National Health Services Board on the Echocardiography School Screening Program for Rheumatic Heart Disease, National Health Services, Samoa 2009.-- Tompkins DG, Boxerbaum B, Liebman J. Long-term prognosis of rheumatic fever patients receiving regular intramuscular benzathine penicillin. Circulation 1972;45:543-551.-- Marijion E, Celermajer DS, Tafflet M, et al. Rheumatic heart disease screening by echocardiography: The inadequacy of the World Health Organization Criteria for optimizing the diagnosis of subclinical disease. Circulation 2009;120:663-668.-- Ramakrishnan S. Echocardiography in acute rheumatic fever. Ann Pediatr Card 2009;2(1):61-4.-- International Standardization of Echocardiographic Diagnosis of Rheumatic Heart Disease Project 2009-2011.-- Tani LY, Veasy G, Minich LA, Shaddy RE. Rheumatic fever in children younger than 5 years: Is the presentation different? Paediatrics 2003;112:1065-68.-

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Acute rheumatic fever results from an autoimmune response to infection with a group A streptococcus resulting in multi-organ involvement., Except for carditis, none of its manifestations lead to permanent damage.Rheumatic fever and its consequences (rheumatic heart disease) remain one of the devastating diseases affecting the Pacific people wherever they are around the world.1-9 There are nearly 16 million people worldwide who suffer from rheumatic heart diseases (RHD). More than 200,000 deaths each year are due to the disease and its sequealae.10 The vast majority of the burden is borne by the developing countries.Acute rheumatic fever (ARF) and RHD are becoming less common in developed countries as living conditions, hygiene, access to medical care, nutrition and socioeconomic standards improve. Unfortunately within some of the developed countries like USA, Australia, New Zealand and Hawaii, the presence of ARF and RHD are still quite prevalent amongst its indigenous and migrant populations particularly those with origins in the Pacific. With ARF and RHD so prevalent amongst the Pacific people living in the islands or in other countries, this suggests a genetic link or predisposition. Unfortunately the genetic link has not yet been established.11Data collection to assess the burden of ARF and RHD in any country is always a challenge because of the history of the disease and the health infrastructure. This is especially true in the Pacific and Samoa. The Jones Diagnostic Criteria for ARF is difficult to apply to rheumatic fever cases in Samoa because people present late, with recurrent symptoms. In our experience, many of our people present at the stage of established RHD rather than at the sore throat and ARF stage.Epidemiology of acute rheumatic fever (ARF) and rheumatic heart disease (RHD)In Samoa the reported incidence of ARF was 16.1 per 100,000 in 1985 and the prevalence of RHD was 2.3 per 1000 in 1986.3 In 2000 the incidence of ARF was 35 per 100,0001 from those who were referred from all the clinics and hospitals with the diagnosis of ARF. In Hawaii the incidence of ARF in Samoans was 206 per 100,000 in 1984.8 The incidence of RHD in Samoa was 66 per 100,000 in 2005 and 68 per 100,000 in 2007 using the Echocardiogram Register.1The Echocardiogram Register recorded everyone who had had an echo study including all those who were diagnosed with ARF, those who were referred because of murmurs, and those who were referred for cardiac assessment for other reasons like heart failure, ischaemic heart disease, and congenital heart diseases. The School Auscultation Survey for Heart Murmurs by Steers and Adams in 199612 implied a RHD prevalence of 77.8 per 1000 in schools. Unfortunately there was no echocardiogram used.In 2000 and 2001, our Rheumatic Fever Team under NZAID funding performed an auscultation study for RHD in schools on 2828 kids from age 5 to age 13. Those who presented with murmurs were referred for echocardiography. The prevalence of heart murmurs was 18 per 1000, and after echocardiography the prevalence of new RHD was 3 per 1000.13,14 ARF and RHD are common in other Pacific countries.Tonga had RHD prevalence in school children of 33.2 per 1000 in 2008, and the prevalence increased with age peaking at 42.6 per 1000 in children 10-12 years old.4 This survey of 5053 of primary school children was initially conducted with auscultation and those with heart murmurs progressed to echocardiography. Auscultation will miss about 20-30% of RHD15 and therefore the actual RHD prevalence may be higher than reported if all the children were scanned.Fiji had an ARF incidence of 15.2 per 100,000 from 2005-2007 in children age 5-15 years.5 The prevalence of RHD in school children age 5-15 years old was 4.1 per 1000 for definite RHD and 8.4 per 1000 for definite and probable RHD.6 This survey of 3462 of primary school children was initially conducted with auscultation and those with heart murmurs progressed to echocardiography.During the 1950s and 1960s there were 20-30 per 100,000 cases of ARF in New Zealand. The prevalence of ARF amongst Pacific people in New Zealand was high. The incidence of ARF in New Zealand had declined to 2.8 per 100,000 from 1995-2000, 1.9% per 100,000 in 2005, and 2.5 per 100,000 in 2006.7,16Pacific people comprise about 30% of cases where ethnicity was recorded and M ori was 62%. The highest rate of ARF was in the 10-14 year old age group amongst the Pacific people (16.1 per 100,000) residing in Auckland. Some of the reported RHD prevalence was 18.6 per 1000 in Cook Island, 8.0 per 1000 in French Polynesia, and 10 per 1000 in New Caledonia.2,3,17As many people in Samoa have relatives in New Zealand there is a high incidence of migration between Samoa and New Zealand every year. This may affect the epidemiology of rheumatic fever and RHD in New Zealand and may have huge implications in the management and the control of this disease.Rheumatic Fever Programme in SamoaThe Rheumatic Fever Programme in Samoa started in the mid-1970s and was re-established in 1984. In 2000 the NZAID organisation assisted with 2-3 year funding which enabled a school-based rheumatic fever prevention pilot programme. In 2006 funding was secured from Vodafone Foundation for establishing a RHD Project from 2007 to 2009 mainly to employ dedicated staff to oversee the Rheumatic Fever Programme. The funding was administered through the World Heart Federation with technical advice from Menzies Research Centre. The Samoan Cabinet and the Ministry of Health appointed Professor S Viali (Dean of the Oceania University of Medicine) as director, RN Puleiala Saena as the nurse, and Vailogoua Futi as the secretary and field assistant.The Project was coordinated from the main National Hospital Tupua Tamasese Meaole (TTM) in the Rheumatic Fever Centre (RFC). People with RF and RHD were seen in the TTM Paediatric Clinic (if <13 years), TTM Medical Clinic (if 226513 years), Tuasivi District Hospital (in Savaii), Safotu District Hospital (Savaii), and the RFC. Echocardiograms were mainly performed by Dr S Viali in adults and kids with RF and RHD in the RFC, and in the Paediatric Clinic by Dr F Fatupaito, and sometimes by Dr L Fiu in the Medical Clinic.The information on people diagnosed with RF or RHD from all centres was regularly collected by our secretary to enter into our database. Intramuscular (IM) penicillin was delivered mainly through the RFC by RN Pule, Paediatric Clinic, Tuasivi District Hospital and Safotu District Hospital. Medical, cardiac and echocardiogram follow-up were mainly done in the RFC, TTM Paediatric Clinic and TTM Medical Clinic.There has been strong focus on public awareness and education regarding ARF and RHD.Multiple workshops on rheumatic fever were carried out for the medical and nursing staff in 2007 and 2008 using local and overseas experts. Mobile clinics and echocardiograms were done by Dr S Viali in the outer villages of Upolu and Savaii, and he was occasionally accompanied by an overseas cardiologist. Several rheumatic fever prevention programmes were frequently aired on national television (TV1 and TV3) between 2007-2009 and many articles on rheumatic fever prevention have been published in several popular local newspapers.NZAID also supported a rheumatic fever primary prevention programme for 2006 and 2007 under the Ministry of Health. This focused on health education and health promotion in rheumatic fever.Rheumatic Fever RegisterRegister-based rheumatic fever programmes have been successful in countries like New Zealand, Australia, India, Cuba and Egypt.7,18-21 To ensure efficient delivery of services and prophylaxis, and to monitor service delivery, it is crucial that the Register be accurate and up-to-date with people with ARF (known and past) and RHD. It is also a very important epidemiological tool. The most important outcome is the improvement of the status of rheumatic fever and rheumatic heart disease control.The Rheumatic Fever Programme in Samoa kept a manual register since 1984 which was described earlier.1 This manual register was well kept from 1984 to 2002 but unfortunately this register was lost. The rheumatic fever work and clinics continued with less coordination between the various centres in both islands looking after rheumatic fever patients. During this time the patients presenting with ARF and RHD were recorded in the hospital health information data base.The recent RHD project provided an electronic rheumatic fever register and all the rheumatic patients have been recorded in this database. The RHD project officially started in the beginning of 2007 hence a lot of the old rheumatic fever and RHD patients that were not known were entered, resulting in large numbers entered in 2007 into the electronic register. There were 133 people recorded with RHD in 1974,22 and 354 recorded with RHD in 1986 3, and about 708 were recorded with RHD in the current register in 2009.\r\n Table 1. Rheumatic Fever Register 2003-2009 \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n Disease\r\n \r\n \r\n \r\n 2003\r\n \r\n \r\n \r\n 2004\r\n \r\n \r\n \r\n 2005\r\n \r\n \r\n \r\n 2006\r\n \r\n \r\n \r\n 2007\r\n \r\n \r\n \r\n 2008\r\n \r\n \r\n \r\n 2009\r\n \r\n \r\n \r\n \r\n \r\n ARF\r\n New RHD\r\n Known RHD & ARF\r\n \r\n \r\n \r\n 4\r\n 121\r\n \r\n \r\n \r\n 3\r\n 87\r\n \r\n \r\n \r\n 7\r\n 46\r\n \r\n \r\n \r\n 3\r\n 43\r\n \r\n \r\n \r\n 23\r\n 63\r\n 188\r\n \r\n \r\n \r\n 13\r\n 55\r\n 20\r\n \r\n \r\n \r\n 17\r\n 49\r\n 11\r\n \r\n \r\n \r\n \r\n \r\n Total\r\n \r\n \r\n \r\n 125\r\n \r\n \r\n \r\n 90\r\n \r\n \r\n \r\n 53\r\n \r\n \r\n \r\n 46\r\n \r\n \r\n \r\n 273\r\n \r\n \r\n \r\n 88\r\n \r\n \r\n \r\n 77\r\n \r\n \r\n \r\n \r\n \r\n ARF=acute rheumatic fever; RHD=rheumatic heart disease. Those with ARF either presented or referred to the clinics from GPs or hospitals and some were admitted to the hospitals with acute symptoms of rheumatic fever. The new RHD were those who were referred because of a heart murmur and were found to have RHD, or were admitted for something else and were found to have RHD. Patients with known diagnosis of ARF or RHD were recorded in the year they were identified. The register recorded 314 people from 2003-2006 and 438 people from 2007-2009 (Table 1), 42% were males, and 6.8% have died. The incidence of ARF in Samoa has steadily reduced from 35 per 100,000 in 2000,1 to 30 per 100,000 in 2005, 12.8 per 100,000 in 2007, 7.3 per 100,000 in 2008 and 9.5 per 100,000 in 2009 (Figure 1). \r\n Figure 1. Incidence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) in Samoa For those who presented with ARF, about half had already developed carditis. This may mean that some of these presentations were of recurrent rheumatic fever instead of their first attack. About 1-3% of those with the first ARF attack presented with carditis and with subsequent attacks 25-75% would develop carditis.23,24 The most common age group that presented with ARF was 10-14 years old (57.7%), followed by 15-19 years old (19.2%) and 5-9 years old (17.2%), and none were recorded below the age of 4. The incidence of RHD per 100,000 has steadily decreased from 66 in 2005, to 40.2 in 2007, 34 in 2008, and to 31.8 in 2009 (Figure 1). The most common age group with RHD was 10-14 years old (35.7%), followed by 15-19 years old (26.8%), followed by 5-9 years old (12.1%) and 20-24 years old (10.2%), with only one RHD case recorded below the age of 4. The villages with the most ARF and RHD were in the Upolu urban areas in Apia (Vaitele, Vaiusu, Siusega, Lotopa, Sinamoga, Faatoia, Vaivase) with low numbers in Savaii. The Register has also shown the many undiagnosed RHD presenting in other ways and without ARF symptoms. It is always assumed that the presence of RHD meant that ARF must have occurred at some point in time, whether it was symptomatic or not. The acquiring of symptoms of ARF depended on many factors. The manifestations of major and minor Jones criteria vary in different countries which led to the original Jones criteria changing to the current form. From the current register, for every patient that had been diagnosed with ARF symptoms, there would be another 3-4 with undiagnosed RHD. Penicillin injections compliance Compliance to IM penicillin from 2001-2006 was estimated to be <50% according to the staff working with rheumatic patients, with many patients declining follow-up visits. Compliance was assessed by the numbers of injections per year per patient. With more focus on rheumatic fever by the Ministry of Health, National Health Services and the new Rheumatic Project, the compliance to IM penicillin improved to 74-84% in the four injection centres (RFC TTM, Paediatric Clinic TTM, Tuasivi District Hospital, Safotu District Hospital) (Figure 2). \r\n Figure 2. Compliance to IM penicillin in the four injection centres Those who did not turn up for their injections were phoned by the nurse. Previously the rheumatic fever nurse would take the penicillin injections to the homes of the rheumatic fever patients when they did not turn up. Due to better access to hospitals and improved transportation infrastructure around the island, it is now considerably easier to get the IM penicillin injections. The key to better compliance was enthusiastic, dedicated staff in the Rheumatic Fever Programme and the reminder phone call to remind the patients of the injections. In Savaii the reminder messages were relayed by health staff in the various villages. RHD cardiac surgery Cardiac surgery is very expensive, and small island economies find it difficult to afford these prices.25,26 Samoa has sent the majority of its cardiac patients to New Zealand for surgery since the 1970s. Prior to 1996 less than 20 RHD operations per year were performed in New Zealand on our patients. From 1997 to 2009 between 20 and 28 RHD operations per year (NZ$28,000-NZ$44,000 per valve operation) were performed (Figure 3). The total cost ranged from NZ$560,000 to NZ$1,300,000 per year (>ST$1.9 million tala per year). \r\n Figure 3. Cardiac surgery 1992-2009 In 2003 there were 184 referrals to New Zealand for treatment including 61 (33.3%) referrals for cardiac surgery. For cardiac surgery in 2003, 41% of these were for RHD, 26% for ischaemic heart disease (IHD), 20% for congenital heart disease (CHD) with 15% for others. The 25 RHD surgeries done in 2003 cost about NZ$1.1 million dollars (>ST$1.65 million tala) 2014a significant dent in the Samoan health budget. In 2008 there were 227 referrals to New Zealand including 62 referrals for cardiac surgery 201434% were for RHD, 40% for IHD, 21% for CHD, and 5% for others. In 2009 there were 288 referrals including 84 referrals for cardiac surgery 201431% were for RHD, 43% for IHD, 18% for congenital heart disease, and 8% for others. Though RHD surgery has declined there is a noticeable rise in IHD surgeries as there is a rise in non-communicable and lifestyle diseases in Samoa and the Pacific. Samoa 2019s overseas treatment scheme funded by the Government of Samoa, increases annually and has peaked at ST$10 million tala in 2009. In addition to the above budget, New Zealand AID annually provides about NZ$500,000. There have been ongoing discussions regarding reducing prices for cardiac surgery for Samoa but the recent recession has deferred these discussions. The alternative was to perform these surgeries in Samoa by visiting cardiac teams. In 2007 the cardiac medical mission started with 10 RHD cases operated in Samoa with no fatalities. In 2008, 14 RHD cases were done in Samoa. Unfortunately 50% developed significant pericardial effusion and 3 patients died in Samoa from cardiac tamponade. There are more cardiac operations planned for 2011. There is no question that it is economical to perform cardiac surgery (valve surgery) in Samoa.. Cardiac surgery in New Zealand is very expensive and Samoa 2019s health budget would not be able to sustain the number of surgeries needed. The key to continued success of the good-will cardiac medical mission would be the selection of low risk cases to do safely in Samoa, because the complex infrastructure needed for post-surgical care is not available. The enthusiasm and commitment of the medical team and everyone involved from New Zealand and Samoa is commendable. Echocardiogram screening for RHD in schools People in Samoa usually do not present with acute rheumatic fever symptoms, similar to Fiji,5but present with RHD symptoms, most commonly heart failure. RHD is the dreaded complication of ARF that may influence the prognosis. This has prompted the screening programme in schools. The main purpose is to quickly identify as many RHD as possible, begin early intervention with penicillin and prevent the progression of the RHD and recurrence of ARF. Most of the early survey in schools for RHD was with auscultation. Many recent RHD surveys have been done with auscultation before progressing to echocardiogram4,6 but there were significant numbers of RHD that were missed. Some surveys were done with echocardiogram which had good identification rates of RHD in asymptomatic people.27 The skill of auscultation is cheaper and more accessible than echocardiogram machines, but the identification rate for RHD using auscultation is far inferior to echocardiography.15 For auscultation, years of experience are required before being able to diagnose with certainty. Echocardiography also requires time to acquire the necessary skills to be able to diagnose adequately.. There is no doubt that echocardiography is superior to auscultation in diagnosing RHD, other heart diseases and normal flow murmurs. Though the role of echocardiography in screening is controversial at present, its specificity and application to the natural history and time course of rheumatic fever is currently being revised. On the other hand our patients in Samoa mostly present to health facilities in the advanced heart disease stage and hence screening with echocardiography in schools will certainly pick up those with significant RHD early. There is a current echocardiogram screening programme for RHD in Primary Schools in Samoa for ages 5-13 years old (year 1 to year 8) which will be completed in another 12 months. To date, 3200 kids have been screened from public and private schools in the urban region of Upolu, and the prevalence rates of RHD were similar to Tonga.4,28 There were more RHD in the public schools compared to private schools, and many children with RHD were living in the urban areas of Upolu. The Rheumatic Fever Register suggested that this screening programme should also involve secondary schools (age 14-19 years). Those that were picked up with RHD received penicillin prophylaxis, since penicillin is very effective in preventing progression of RHD.29 The definition of RHD is based on the new diagnostic criteria (morphological changes and Doppler findings).30-32 Summary The incidence of rheumatic fever and rheumatic heart disease in Samoa has decreased. Though it is still 5 times higher than the incidence in New Zealand, it is similar to the situation in Tonga and Fiji. Disease patterns have changed from the early 1900s from infectious disease to non-communicable diseases. Living standards and socioeconomic status have certainly improved which could explain the decline in the incidence of ARF and RHD. None of the ARF presented under the age of 4. About 5% of ARF could occur below the age of 5 years 33. The current rheumatic fever programme is making a difference to the follow-up of patients with ARF and RHD, and the compliance to the IM penicillin prophylaxis programme. The compliance has certainly improved with simple and inexpensive measures. The prevention programmes have also been strengthened. These measures may have also contributed to the decreasing incidence of this disease. The costs of RHD are very high for a small country like Samoa and it is not sustainable in the long term, hence the prevention of the development of ARF and early identification of RHD are some of the important strategies to reduce the burden and suffering of RHD. It is also unfortunate that many of our patients do not present with acute symptoms but with established RHD, hence screening for RHD in schools may be the most effective way of reducing the overall burden of RHD. By treating the many undiagnosed RHD with penicillin, this will prevent the progression of cardiac valve damage thus reducing suffering to the patients and the cost for cardiac surgery.\r\n \r\n \r\n \r\n \r\n

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Satupaitea Viali, Specialist Physician & Cardiologist, Director of Rheumatic Fever Project, Medical Specialist Clinic & National Health Services; Puleiala Saena, Rheumatic Fever Nurse, National Health Services; Vailogoua Futi, Secretary, National Health Services; Apia, Samoa

Acknowledgements

Correspondence

Dr Satupaitea Viali (MPH, FRACP, FCSANZ), Medical Specialist Clinic, PO Box 2122, Apia, Samoa

Correspondence Email

satu.viali@gmail.com

Competing Interests

None known

'- Viali S. Rheumatic Fever and Rheumatic Heart Disease in Samoa. Pacific Health Dialogue 2006;13(2):31-38.-- Steer A, Colquhuon S, Viali S, et al. Control of Rheumatic Heart Disease in the Pacific Region. Pacific Health Dialogue 2006:13(2):49-55.-- Neutze JM. Rheumatic fever and rheumatic heart disease in the Western Pacific region. NZ Med J 1988:101:404-406.-- Carapedis J, Hardy M, Fakakovikaetau T, et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan school children. Nature Review Cardiology 2008;(5),411-417.-- Steers AC, Kado J, Jenny AW, et al. Acute rheumatic fever and rheumatic heart disease in Fiji: prospective surveillance, 2005-2007. MJA 2009;190(3):133-135.-- Steers AC, Kado J, Wilson J, et al. High prevalence of rheumatic heart disease by clinical and echocardiographic screening among children in Fiji. J Heart Valve Disease 2009;18(3):336.-- Thornley C, McNicholas A, Baker M, Lennon D. Rheumatic Fever Registers in New Zealand. New Zealand Public Health Report 2001:8(6):41-44.-- Chun RT, Reddy V, Rhoads GG. Occurrence and prevention of rheumatic fever among ethnic groups of Hawaii. Am J Dis Child 1984;138(5):476-478.-- Miyake CY, Gauvreau K, Tani LY, et al. Characteristics of children discharged from hospitals in the United States in 2000 with the diagnosis of Acute Rheumatic Fever. Paediatrics 2007;120;503-508.-- Carapedis J, McDonald M, Wilson N. Acute rheumatic fever. Lancet 2005;366:155-168.-- Bryant PA, Robins-Browne R, Carapedis JR, Curtis N. Some of the people, some of the time: Susceptibility to Acute Rheumatic Fever. Circulation 2009;119;742-753.-- Steer AC, Adams J, Carlin J, et al. Rheumatic heart disease in school children in Samoa. Archives of Diseases in Childhood 1999;81(4):373.-- Viali S. Echocardiogram findings on children with murmurs picked up by the School-based Rheumatic Fever Prevention program. A preliminary report to the Ministry of Health, Samoa. 2001.-- McCleland V. Final Report - Process Evaluation of school based rheumatic fever prevention pilot programme. Ministry of Health, Samoa. 2002.-- Abernethy M, Bass N, et al. Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever. Aust NZ J Med 1994;24:530-35.-- ESR. Notifiable and other diseases in New Zealand: Annual report 2006. Wellington: ESR; 2007-- Rheumatic fever and rheumatic heart disease. WHO 1988.-- Strasser T, Dondog N, El Kholy A, et al. The community control of rheumatic fever and rheumatic heart disease: a report of a WHO international cooperative project. Bull World Health Organ 1981;59:285-294.-- Kumar R, Raizada A, Aggarwal AK, et al. A community-based rheumatic fever / rheumatic heart disease cohort: twelve-year experience. Indian Heart Journal 2002:54:54-58.-- World Health Organization. Rheumatic fever and rheumatic heart disease: report of a WHO expert consultation. Geneva: WHO; 2004.-- Noonan S, Edmond K, Krause V, et al. The top end rheumatic heart disease control program I. Report on progress. NT Dis Control Bull 2001;8:15-18.-- Smith WM. An appraisal of rheumatic valvular heart disease in Western Samoa. Aust New Zealand J Med 1979;9:560-65.-- Siegel AC, Johnson EE, Stollerman GH. Controlled studies of streptococcal pharyngitis in a paediatric population, 1: factors related to the attack rate of rheumatic fever. NEJM 1961;265;559-565.-- Manyemba J, Mayosi BM. Penicillin for secondary prevention of rheumatic fever. Cochrane Database Syst Rev 2002;(3):CD002227.-- Viali S. Cardiology and Cardio-surgical Report to the Ministry of Health, Samoa. 2003.-- Viali S. Clinical Medical Audit Presentation. Samoa Medical Association Medical Seminar, Apia, Samoa; 2004.-- Marijion E, Ou P, Celermajer DS, Ferreira B, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. NEJM 2007;357:470-476.-- Viali S. Report to the National Health Services Board on the Echocardiography School Screening Program for Rheumatic Heart Disease, National Health Services, Samoa 2009.-- Tompkins DG, Boxerbaum B, Liebman J. Long-term prognosis of rheumatic fever patients receiving regular intramuscular benzathine penicillin. Circulation 1972;45:543-551.-- Marijion E, Celermajer DS, Tafflet M, et al. Rheumatic heart disease screening by echocardiography: The inadequacy of the World Health Organization Criteria for optimizing the diagnosis of subclinical disease. Circulation 2009;120:663-668.-- Ramakrishnan S. Echocardiography in acute rheumatic fever. Ann Pediatr Card 2009;2(1):61-4.-- International Standardization of Echocardiographic Diagnosis of Rheumatic Heart Disease Project 2009-2011.-- Tani LY, Veasy G, Minich LA, Shaddy RE. Rheumatic fever in children younger than 5 years: Is the presentation different? Paediatrics 2003;112:1065-68.-

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Acute rheumatic fever results from an autoimmune response to infection with a group A streptococcus resulting in multi-organ involvement., Except for carditis, none of its manifestations lead to permanent damage.Rheumatic fever and its consequences (rheumatic heart disease) remain one of the devastating diseases affecting the Pacific people wherever they are around the world.1-9 There are nearly 16 million people worldwide who suffer from rheumatic heart diseases (RHD). More than 200,000 deaths each year are due to the disease and its sequealae.10 The vast majority of the burden is borne by the developing countries.Acute rheumatic fever (ARF) and RHD are becoming less common in developed countries as living conditions, hygiene, access to medical care, nutrition and socioeconomic standards improve. Unfortunately within some of the developed countries like USA, Australia, New Zealand and Hawaii, the presence of ARF and RHD are still quite prevalent amongst its indigenous and migrant populations particularly those with origins in the Pacific. With ARF and RHD so prevalent amongst the Pacific people living in the islands or in other countries, this suggests a genetic link or predisposition. Unfortunately the genetic link has not yet been established.11Data collection to assess the burden of ARF and RHD in any country is always a challenge because of the history of the disease and the health infrastructure. This is especially true in the Pacific and Samoa. The Jones Diagnostic Criteria for ARF is difficult to apply to rheumatic fever cases in Samoa because people present late, with recurrent symptoms. In our experience, many of our people present at the stage of established RHD rather than at the sore throat and ARF stage.Epidemiology of acute rheumatic fever (ARF) and rheumatic heart disease (RHD)In Samoa the reported incidence of ARF was 16.1 per 100,000 in 1985 and the prevalence of RHD was 2.3 per 1000 in 1986.3 In 2000 the incidence of ARF was 35 per 100,0001 from those who were referred from all the clinics and hospitals with the diagnosis of ARF. In Hawaii the incidence of ARF in Samoans was 206 per 100,000 in 1984.8 The incidence of RHD in Samoa was 66 per 100,000 in 2005 and 68 per 100,000 in 2007 using the Echocardiogram Register.1The Echocardiogram Register recorded everyone who had had an echo study including all those who were diagnosed with ARF, those who were referred because of murmurs, and those who were referred for cardiac assessment for other reasons like heart failure, ischaemic heart disease, and congenital heart diseases. The School Auscultation Survey for Heart Murmurs by Steers and Adams in 199612 implied a RHD prevalence of 77.8 per 1000 in schools. Unfortunately there was no echocardiogram used.In 2000 and 2001, our Rheumatic Fever Team under NZAID funding performed an auscultation study for RHD in schools on 2828 kids from age 5 to age 13. Those who presented with murmurs were referred for echocardiography. The prevalence of heart murmurs was 18 per 1000, and after echocardiography the prevalence of new RHD was 3 per 1000.13,14 ARF and RHD are common in other Pacific countries.Tonga had RHD prevalence in school children of 33.2 per 1000 in 2008, and the prevalence increased with age peaking at 42.6 per 1000 in children 10-12 years old.4 This survey of 5053 of primary school children was initially conducted with auscultation and those with heart murmurs progressed to echocardiography. Auscultation will miss about 20-30% of RHD15 and therefore the actual RHD prevalence may be higher than reported if all the children were scanned.Fiji had an ARF incidence of 15.2 per 100,000 from 2005-2007 in children age 5-15 years.5 The prevalence of RHD in school children age 5-15 years old was 4.1 per 1000 for definite RHD and 8.4 per 1000 for definite and probable RHD.6 This survey of 3462 of primary school children was initially conducted with auscultation and those with heart murmurs progressed to echocardiography.During the 1950s and 1960s there were 20-30 per 100,000 cases of ARF in New Zealand. The prevalence of ARF amongst Pacific people in New Zealand was high. The incidence of ARF in New Zealand had declined to 2.8 per 100,000 from 1995-2000, 1.9% per 100,000 in 2005, and 2.5 per 100,000 in 2006.7,16Pacific people comprise about 30% of cases where ethnicity was recorded and M ori was 62%. The highest rate of ARF was in the 10-14 year old age group amongst the Pacific people (16.1 per 100,000) residing in Auckland. Some of the reported RHD prevalence was 18.6 per 1000 in Cook Island, 8.0 per 1000 in French Polynesia, and 10 per 1000 in New Caledonia.2,3,17As many people in Samoa have relatives in New Zealand there is a high incidence of migration between Samoa and New Zealand every year. This may affect the epidemiology of rheumatic fever and RHD in New Zealand and may have huge implications in the management and the control of this disease.Rheumatic Fever Programme in SamoaThe Rheumatic Fever Programme in Samoa started in the mid-1970s and was re-established in 1984. In 2000 the NZAID organisation assisted with 2-3 year funding which enabled a school-based rheumatic fever prevention pilot programme. In 2006 funding was secured from Vodafone Foundation for establishing a RHD Project from 2007 to 2009 mainly to employ dedicated staff to oversee the Rheumatic Fever Programme. The funding was administered through the World Heart Federation with technical advice from Menzies Research Centre. The Samoan Cabinet and the Ministry of Health appointed Professor S Viali (Dean of the Oceania University of Medicine) as director, RN Puleiala Saena as the nurse, and Vailogoua Futi as the secretary and field assistant.The Project was coordinated from the main National Hospital Tupua Tamasese Meaole (TTM) in the Rheumatic Fever Centre (RFC). People with RF and RHD were seen in the TTM Paediatric Clinic (if <13 years), TTM Medical Clinic (if 226513 years), Tuasivi District Hospital (in Savaii), Safotu District Hospital (Savaii), and the RFC. Echocardiograms were mainly performed by Dr S Viali in adults and kids with RF and RHD in the RFC, and in the Paediatric Clinic by Dr F Fatupaito, and sometimes by Dr L Fiu in the Medical Clinic.The information on people diagnosed with RF or RHD from all centres was regularly collected by our secretary to enter into our database. Intramuscular (IM) penicillin was delivered mainly through the RFC by RN Pule, Paediatric Clinic, Tuasivi District Hospital and Safotu District Hospital. Medical, cardiac and echocardiogram follow-up were mainly done in the RFC, TTM Paediatric Clinic and TTM Medical Clinic.There has been strong focus on public awareness and education regarding ARF and RHD.Multiple workshops on rheumatic fever were carried out for the medical and nursing staff in 2007 and 2008 using local and overseas experts. Mobile clinics and echocardiograms were done by Dr S Viali in the outer villages of Upolu and Savaii, and he was occasionally accompanied by an overseas cardiologist. Several rheumatic fever prevention programmes were frequently aired on national television (TV1 and TV3) between 2007-2009 and many articles on rheumatic fever prevention have been published in several popular local newspapers.NZAID also supported a rheumatic fever primary prevention programme for 2006 and 2007 under the Ministry of Health. This focused on health education and health promotion in rheumatic fever.Rheumatic Fever RegisterRegister-based rheumatic fever programmes have been successful in countries like New Zealand, Australia, India, Cuba and Egypt.7,18-21 To ensure efficient delivery of services and prophylaxis, and to monitor service delivery, it is crucial that the Register be accurate and up-to-date with people with ARF (known and past) and RHD. It is also a very important epidemiological tool. The most important outcome is the improvement of the status of rheumatic fever and rheumatic heart disease control.The Rheumatic Fever Programme in Samoa kept a manual register since 1984 which was described earlier.1 This manual register was well kept from 1984 to 2002 but unfortunately this register was lost. The rheumatic fever work and clinics continued with less coordination between the various centres in both islands looking after rheumatic fever patients. During this time the patients presenting with ARF and RHD were recorded in the hospital health information data base.The recent RHD project provided an electronic rheumatic fever register and all the rheumatic patients have been recorded in this database. The RHD project officially started in the beginning of 2007 hence a lot of the old rheumatic fever and RHD patients that were not known were entered, resulting in large numbers entered in 2007 into the electronic register. There were 133 people recorded with RHD in 1974,22 and 354 recorded with RHD in 1986 3, and about 708 were recorded with RHD in the current register in 2009.\r\n Table 1. Rheumatic Fever Register 2003-2009 \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n \r\n Disease\r\n \r\n \r\n \r\n 2003\r\n \r\n \r\n \r\n 2004\r\n \r\n \r\n \r\n 2005\r\n \r\n \r\n \r\n 2006\r\n \r\n \r\n \r\n 2007\r\n \r\n \r\n \r\n 2008\r\n \r\n \r\n \r\n 2009\r\n \r\n \r\n \r\n \r\n \r\n ARF\r\n New RHD\r\n Known RHD & ARF\r\n \r\n \r\n \r\n 4\r\n 121\r\n \r\n \r\n \r\n 3\r\n 87\r\n \r\n \r\n \r\n 7\r\n 46\r\n \r\n \r\n \r\n 3\r\n 43\r\n \r\n \r\n \r\n 23\r\n 63\r\n 188\r\n \r\n \r\n \r\n 13\r\n 55\r\n 20\r\n \r\n \r\n \r\n 17\r\n 49\r\n 11\r\n \r\n \r\n \r\n \r\n \r\n Total\r\n \r\n \r\n \r\n 125\r\n \r\n \r\n \r\n 90\r\n \r\n \r\n \r\n 53\r\n \r\n \r\n \r\n 46\r\n \r\n \r\n \r\n 273\r\n \r\n \r\n \r\n 88\r\n \r\n \r\n \r\n 77\r\n \r\n \r\n \r\n \r\n \r\n ARF=acute rheumatic fever; RHD=rheumatic heart disease. Those with ARF either presented or referred to the clinics from GPs or hospitals and some were admitted to the hospitals with acute symptoms of rheumatic fever. The new RHD were those who were referred because of a heart murmur and were found to have RHD, or were admitted for something else and were found to have RHD. Patients with known diagnosis of ARF or RHD were recorded in the year they were identified. The register recorded 314 people from 2003-2006 and 438 people from 2007-2009 (Table 1), 42% were males, and 6.8% have died. The incidence of ARF in Samoa has steadily reduced from 35 per 100,000 in 2000,1 to 30 per 100,000 in 2005, 12.8 per 100,000 in 2007, 7.3 per 100,000 in 2008 and 9.5 per 100,000 in 2009 (Figure 1). \r\n Figure 1. Incidence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) in Samoa For those who presented with ARF, about half had already developed carditis. This may mean that some of these presentations were of recurrent rheumatic fever instead of their first attack. About 1-3% of those with the first ARF attack presented with carditis and with subsequent attacks 25-75% would develop carditis.23,24 The most common age group that presented with ARF was 10-14 years old (57.7%), followed by 15-19 years old (19.2%) and 5-9 years old (17.2%), and none were recorded below the age of 4. The incidence of RHD per 100,000 has steadily decreased from 66 in 2005, to 40.2 in 2007, 34 in 2008, and to 31.8 in 2009 (Figure 1). The most common age group with RHD was 10-14 years old (35.7%), followed by 15-19 years old (26.8%), followed by 5-9 years old (12.1%) and 20-24 years old (10.2%), with only one RHD case recorded below the age of 4. The villages with the most ARF and RHD were in the Upolu urban areas in Apia (Vaitele, Vaiusu, Siusega, Lotopa, Sinamoga, Faatoia, Vaivase) with low numbers in Savaii. The Register has also shown the many undiagnosed RHD presenting in other ways and without ARF symptoms. It is always assumed that the presence of RHD meant that ARF must have occurred at some point in time, whether it was symptomatic or not. The acquiring of symptoms of ARF depended on many factors. The manifestations of major and minor Jones criteria vary in different countries which led to the original Jones criteria changing to the current form. From the current register, for every patient that had been diagnosed with ARF symptoms, there would be another 3-4 with undiagnosed RHD. Penicillin injections compliance Compliance to IM penicillin from 2001-2006 was estimated to be <50% according to the staff working with rheumatic patients, with many patients declining follow-up visits. Compliance was assessed by the numbers of injections per year per patient. With more focus on rheumatic fever by the Ministry of Health, National Health Services and the new Rheumatic Project, the compliance to IM penicillin improved to 74-84% in the four injection centres (RFC TTM, Paediatric Clinic TTM, Tuasivi District Hospital, Safotu District Hospital) (Figure 2). \r\n Figure 2. Compliance to IM penicillin in the four injection centres Those who did not turn up for their injections were phoned by the nurse. Previously the rheumatic fever nurse would take the penicillin injections to the homes of the rheumatic fever patients when they did not turn up. Due to better access to hospitals and improved transportation infrastructure around the island, it is now considerably easier to get the IM penicillin injections. The key to better compliance was enthusiastic, dedicated staff in the Rheumatic Fever Programme and the reminder phone call to remind the patients of the injections. In Savaii the reminder messages were relayed by health staff in the various villages. RHD cardiac surgery Cardiac surgery is very expensive, and small island economies find it difficult to afford these prices.25,26 Samoa has sent the majority of its cardiac patients to New Zealand for surgery since the 1970s. Prior to 1996 less than 20 RHD operations per year were performed in New Zealand on our patients. From 1997 to 2009 between 20 and 28 RHD operations per year (NZ$28,000-NZ$44,000 per valve operation) were performed (Figure 3). The total cost ranged from NZ$560,000 to NZ$1,300,000 per year (>ST$1.9 million tala per year). \r\n Figure 3. Cardiac surgery 1992-2009 In 2003 there were 184 referrals to New Zealand for treatment including 61 (33.3%) referrals for cardiac surgery. For cardiac surgery in 2003, 41% of these were for RHD, 26% for ischaemic heart disease (IHD), 20% for congenital heart disease (CHD) with 15% for others. The 25 RHD surgeries done in 2003 cost about NZ$1.1 million dollars (>ST$1.65 million tala) 2014a significant dent in the Samoan health budget. In 2008 there were 227 referrals to New Zealand including 62 referrals for cardiac surgery 201434% were for RHD, 40% for IHD, 21% for CHD, and 5% for others. In 2009 there were 288 referrals including 84 referrals for cardiac surgery 201431% were for RHD, 43% for IHD, 18% for congenital heart disease, and 8% for others. Though RHD surgery has declined there is a noticeable rise in IHD surgeries as there is a rise in non-communicable and lifestyle diseases in Samoa and the Pacific. Samoa 2019s overseas treatment scheme funded by the Government of Samoa, increases annually and has peaked at ST$10 million tala in 2009. In addition to the above budget, New Zealand AID annually provides about NZ$500,000. There have been ongoing discussions regarding reducing prices for cardiac surgery for Samoa but the recent recession has deferred these discussions. The alternative was to perform these surgeries in Samoa by visiting cardiac teams. In 2007 the cardiac medical mission started with 10 RHD cases operated in Samoa with no fatalities. In 2008, 14 RHD cases were done in Samoa. Unfortunately 50% developed significant pericardial effusion and 3 patients died in Samoa from cardiac tamponade. There are more cardiac operations planned for 2011. There is no question that it is economical to perform cardiac surgery (valve surgery) in Samoa.. Cardiac surgery in New Zealand is very expensive and Samoa 2019s health budget would not be able to sustain the number of surgeries needed. The key to continued success of the good-will cardiac medical mission would be the selection of low risk cases to do safely in Samoa, because the complex infrastructure needed for post-surgical care is not available. The enthusiasm and commitment of the medical team and everyone involved from New Zealand and Samoa is commendable. Echocardiogram screening for RHD in schools People in Samoa usually do not present with acute rheumatic fever symptoms, similar to Fiji,5but present with RHD symptoms, most commonly heart failure. RHD is the dreaded complication of ARF that may influence the prognosis. This has prompted the screening programme in schools. The main purpose is to quickly identify as many RHD as possible, begin early intervention with penicillin and prevent the progression of the RHD and recurrence of ARF. Most of the early survey in schools for RHD was with auscultation. Many recent RHD surveys have been done with auscultation before progressing to echocardiogram4,6 but there were significant numbers of RHD that were missed. Some surveys were done with echocardiogram which had good identification rates of RHD in asymptomatic people.27 The skill of auscultation is cheaper and more accessible than echocardiogram machines, but the identification rate for RHD using auscultation is far inferior to echocardiography.15 For auscultation, years of experience are required before being able to diagnose with certainty. Echocardiography also requires time to acquire the necessary skills to be able to diagnose adequately.. There is no doubt that echocardiography is superior to auscultation in diagnosing RHD, other heart diseases and normal flow murmurs. Though the role of echocardiography in screening is controversial at present, its specificity and application to the natural history and time course of rheumatic fever is currently being revised. On the other hand our patients in Samoa mostly present to health facilities in the advanced heart disease stage and hence screening with echocardiography in schools will certainly pick up those with significant RHD early. There is a current echocardiogram screening programme for RHD in Primary Schools in Samoa for ages 5-13 years old (year 1 to year 8) which will be completed in another 12 months. To date, 3200 kids have been screened from public and private schools in the urban region of Upolu, and the prevalence rates of RHD were similar to Tonga.4,28 There were more RHD in the public schools compared to private schools, and many children with RHD were living in the urban areas of Upolu. The Rheumatic Fever Register suggested that this screening programme should also involve secondary schools (age 14-19 years). Those that were picked up with RHD received penicillin prophylaxis, since penicillin is very effective in preventing progression of RHD.29 The definition of RHD is based on the new diagnostic criteria (morphological changes and Doppler findings).30-32 Summary The incidence of rheumatic fever and rheumatic heart disease in Samoa has decreased. Though it is still 5 times higher than the incidence in New Zealand, it is similar to the situation in Tonga and Fiji. Disease patterns have changed from the early 1900s from infectious disease to non-communicable diseases. Living standards and socioeconomic status have certainly improved which could explain the decline in the incidence of ARF and RHD. None of the ARF presented under the age of 4. About 5% of ARF could occur below the age of 5 years 33. The current rheumatic fever programme is making a difference to the follow-up of patients with ARF and RHD, and the compliance to the IM penicillin prophylaxis programme. The compliance has certainly improved with simple and inexpensive measures. The prevention programmes have also been strengthened. These measures may have also contributed to the decreasing incidence of this disease. The costs of RHD are very high for a small country like Samoa and it is not sustainable in the long term, hence the prevention of the development of ARF and early identification of RHD are some of the important strategies to reduce the burden and suffering of RHD. It is also unfortunate that many of our patients do not present with acute symptoms but with established RHD, hence screening for RHD in schools may be the most effective way of reducing the overall burden of RHD. By treating the many undiagnosed RHD with penicillin, this will prevent the progression of cardiac valve damage thus reducing suffering to the patients and the cost for cardiac surgery.\r\n \r\n \r\n \r\n \r\n

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Satupaitea Viali, Specialist Physician & Cardiologist, Director of Rheumatic Fever Project, Medical Specialist Clinic & National Health Services; Puleiala Saena, Rheumatic Fever Nurse, National Health Services; Vailogoua Futi, Secretary, National Health Services; Apia, Samoa

Acknowledgements

Correspondence

Dr Satupaitea Viali (MPH, FRACP, FCSANZ), Medical Specialist Clinic, PO Box 2122, Apia, Samoa

Correspondence Email

satu.viali@gmail.com

Competing Interests

None known

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