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Current Situation of Acute Rheumatic Fever and Rheumatic Heart Disease in Latin America and the Caribbean: A Systematic Review Cover

Current Situation of Acute Rheumatic Fever and Rheumatic Heart Disease in Latin America and the Caribbean: A Systematic Review

Global Heart
Volume 17 (2022): Issue 1
By: Maria Alejandra Jaimes-Reyes,  Manuel Urina-Jassir,  Manuel Urina-Triana and  Miguel Urina-Triana  
Open Access
|Sep 2022
Figures & tables

Figures & Tables

Figure 1

PRISMA [11] flow diagram for study selection.

Table 1

Prevalence and incidence of acute rheumatic fever and rheumatic heart disease in Latin America and the Caribbean.

REFERENCECOUNTRYSETTINGTARGET POPULATIONDIAGNOSTIC CRITERIAPERIODPREVALENCEINCIDENCE
Acute Rheumatic Fever
Noah, 1994 [47]BarbadosPopulationChildren TotalJones1971–1990NRTotal population:
1971–1972: 13/100,000
1973: 12/100,000
1974: 5/100,000
1975: 8/100,000
1976–1977: 7/100,000
1978: 9/100,000
1979: 8/100,000
1980: 5/100,000
1981: 3/100,000
1982–1984: 5/100,000
1985: 3/100,000
1986–1990: 2/100,000

Childhood population (<19 years)‘Since 1986’: 8/100,000
Alves Meira et al., 1995 [18]BrazilSchool10–20 yearsJones19923.6/1,000NR
Berrios et al., 1993 [21]ChileCommunityN/AJones1982–1986NR1982–1985: 22.5 per year
1986(6–14-years): 21.7/100,000
Luque et al., 2006 [38]ChilePopulationN/AN/A1978–1998NR1978: 2.2/100,000
1979: 3.2/100,000
1980: 1.4/100,000
1981: 1.6/100,000
1982: 2.4/100,000
1983: 3/100,000
1984: 2.5/100,000
1985: 2/100,000
1986: 1.9/100,000
1987: 1.3/100,000
1988–89: 1/100,000
1990: 0.6/100,000
1991–92: 0.5/100,000
1993: 0.3/100,000
1994–95: 0.2/100,000
1996–97: 0.1/100,000
1998: 0/100,000
Nordet et al., 2008 [48]CubaSchool5–14 yearsInactive RF: ‘History of ARF without established RHD’1985, 19961985: 1.75/1,000
1996: 5.78/1,000		See below in ARF/RHD section for incidence
Bach et al., 1996 [19]Martinique GuadeloupeSchool Hospital<20 yearsJones1982–1983NRMartinique: 19.6/100,000
Guadeloupe: 17.4/100,000
Soto Lopez et al., 2001 [58]MexicoPopulation5–20 yearsJones1994–1999NR‘Annual incidence tendency decreased from 1.3% to 0.3%’
Rheumatic Heart Disease
Meira et al., 2005 [39]BrazilHospitalChildren AdolescentEcho21983–1998NR186 (72.1%) – Severe: 41 (15.9%) out of 258 with ARF
Miranda et al., 2014 [43]BrazilSchoolChildren AdolescentAuscultation Echo (WHO)2010–2011Clinical:
AR: 3.7/1,000
MR: 3.7/1,000

Echo:
AR: 7.5/1,000
MR: 18.7/1,000		NR
Nascimento et al., 2018 [46]BrazilSchool Primary care centersChildren AdolescentEcho (WHF)2014–2016Borderline RHD:
4% (478/12,048)
Definite RHD:
0.5% (63/12,048)		NR
Nascimento et al., 2021 [45]BrazilCommunityPregnantEcho (ASE-REWARD study)2018–2019Hand-held echo screening: 3.2% (36/1,112)
Standard echo: 1.2% (12/1,112)		NR
Nordet et al., 2008 [48]CubaSchool5–14 yearsEcho31985, 19961985: 2.27/1,000
1996: 0.24/1,000		See below in ARF/RHD section for incidence
Paar et al., 2010 [51]NicaraguaCommunityChildren AdultEcho (WHO)2006–2009Pediatric: 48/1,000

Adult: 22/1,000		NR
Spitzer et al., 2015 [60]PeruSchoolChildren AdolescentEcho (WHO & WHF)2014WHO: 19.7/1,000 children

WHF: 3.9/1,000 children		NR
Acute Rheumatic Fever/Rheumatic Heart Disease1
Souza et al., 1990 [59]BrazilSchool CommunityChildren AdolescentJonesN/A20.3% (198/972)NR
WHO Cardiovascular Diseases Unit, 19924 [64]Bolivia El Salvador Jamaica (Americas)SchoolChildrenN/A1986–1990Americas:1.5 (0.1–7.9)/1,000

Bolivia: 7.9/1,000		NR
Nordet et al., 2008 [48]CubaPopulation (Incidence)School (Prevalence)5–25 yearsInactive RF: ‘History of ARF without heart valve damage’RHD: Echo31986, 1996, 20025–14 years:
1985: 8.01/1,000
1996: 1.99/1,000		5–25 years:
1986: 18.6/100,000
1996: 2.5/100,000
2002: 2.4/100,000

5–14 years
1986: 28.4/100,000
1996: 2.7/100,000
2002: 2.8/100,000

[i] 1 Studies that reported the epidemiologic data combining both terms ARF and RHD or referred to them as ‘ARF/RHD’.

2 Reported as ‘the Doppler echocardiography criteria adopted by the echo lab of Universidad Federal Minas Gerais’ [39].

3 Reported as ‘typical RHD valve damage supported by echocardiogram’ [48].

4 The manuscript includes data from 16 countries divided into 5 regions; only data of the Americas region was extracted.

Abbreviations: ASE: American Society of Echocardiography; AR: Aortic regurgitation; ARF: Acute rheumatic fever; ICD: International Classification of Diseases; MR: Mitral regurgitation; N/A: Not available; NR: Not reported; RF: Rheumatic fever; RHD: Rheumatic heart disease; WHF: World Heart Federation; WHO: World Health Organization.

Table 2

Admissions-based data of acute rheumatic fever and rheumatic heart disease in Latin America and the Caribbean.

REFERENCECOUNTRYTARGET POPULATIONPERIODDIAGNOSTIC CRITERIAN/N (%)DESCRIPTION
Acute Rheumatic Fever
Silva et al., 2010 [57]BrazilChildren Adolescent1986, 1991, 1996, 2001, 2006Jones1986: 59/4206 (1.4%)
1991: 17/5206 (0.3%)
1996: 8/5196 (0.15%)
2001: 12/6777(0.18%)
2006: 3/8203 (0.04%)		# of ARF admissions/# of admissions in each period in a single pediatric center
de Araújo Fonseca et al., 2020 [26]BrazilN/A2008–2017ICD-1042,720/11,345,821 (0.4%)# of ARF admissions/# of CVD admissions in Brazil 2008–2017
Defilló Ricart et al., 1991 [27]Dominican RepublicChildren1969–1989Jones121/19,483 (0.62%)# of ARF cases/# of admissions in Cardiology Department of Pediatric Hospital
Stokes Baltazar, 2007 [61]GuatemalaChildren AdolescentAdult2000–2005Jones246/3422 (7.1%)# of ARF cases/# of admissions from a single center
Millard-Bullock, 2012 [42]JamaicaChildren1975–19851989–1995Jones1975–1985: 54% (total pop.: 1079)
1989–1995: 55% (total pop.: 512)		% of patients with ARF among children admitted to hospitals in Jamaica (1975–1985: 4 hospitals, 1989–1995: 3 hospitals)
Soto Lopez et al., 2001 [58]MexicoChildren Adolescent1994–1999JonesIncidence: 6.6 per 1,000

(Total pop.: 3392)		Incidence of new ARF cases out of the total admissions among 5–20-year-olds in a single Cardiology center
Giachetto et al., 1994 [31]UruguayChildren Adolescent1990–1993Jones1990: 14/1731 (0.82%)
1991: 8/2032 (0.39%)
1992: 18/2063 (0.87%)
1993: 18/2256 (0.79%)Total: 58/8,082 (0.71%)		# of ARF admissions/# of children aged 2–14 admissions in a single pediatric center
Rheumatic Heart Disease
Haddad and Bittar, 2005 [32]BrazilN/A1988–2003ICD-9 (1988–94)ICD-10Men: 3.1%
Women: 9.8%		Mean relative percentage per month of RHD diagnosis out of the total admissions in a single CVD center
de Araújo Fonseca et al., 2020 [26]BrazilN/A2008–2017ICD-1078,966/11,345,821 (0.7%)# of RHD admissions/# of CVD admissions in Brazil 2008–2017
Acute Rheumatic Fever/Rheumatic Heart Disease1
Salinas Mondragón et al., 1995 [54]PeruChildren Adolescent1989–1993Jones1989: 9/174 (5.1%)
1990: 10/215 (4.6%)
1991: 16/177 (9.0%)
1992: 15/263 (5.7%)
1993: 16/245 (6.5%)
Total: 66/1074 (6.1%)		# of hospital discharges with ARF/RHD/# discharges in a single pediatric center

[i] 1 Studies that reported the epidemiologic data combining both terms ARF and RHD or referred to them as ‘ARF/RHD’.

Abbreviations: ARF: Acute rheumatic fever; CVD: cardiovascular diseases; ICD: International Classification of Diseases; N/A: Not available; RHD: Rheumatic heart disease.

Table 3

Burden of rheumatic heart disease in Latin America and the Caribbean*.

COMPLICATIONREPORTED DATA AND REFERENCES
General studies1Intervention-only studies1
MortalityRates
BR:
2.6/100,000 women (1986) [37]
1.58/100,000 women (1991–1995) [33]
5.77 (1998), 8.22 (2016) [30]

VE: 7.06 (1955), 3.04 (1966), 0.78 (1975), 1.66 (1985), 1.05 (1994)/100,000 [34]

Proportions
BR:
0.8% (2007–2011) [49]
6.2% (2010–2019) [63]

PE: 6% (1989–1993) [54]		Operative:
BR:
0% (1994–2005) [56]
2.7% (1996–2005) [62]
13% (2008–2009) [22]

CL: 9.4% (1990–2004) [55]

In-hospital or <30 days:
BR:
5.4% (1991–1994) [35]
0% (1994–2005) [56]
9% (2002–2005) [53]
19.2% (2007–2011) [29]
10% (2010–2011) [52]
7.8% (2013–2014) [24]
3.51% (2010–2015) [40]

Follow-up:
BR:
2-month: 0% (2011–2017) [25]
3-month: 0% (2010–2012) [28]
1-year: 0% (2013–2014) [24]
38.5–41.1-month: 7.3% (1991–1994) [35]
63 ± 39-month: 2.9% (1994–2005) [56]

CL: 6.67–7.89-years: 17.7% (1990–2004) [55]

Overall
BR:
0.6% (1987–2010) [41]
8.2% (1996–2005) [62]

MX: 20% [65]
Need for intervention2At baseline
BR:
27% (2007–2011) [49]
25% (2010–2019) [63]

During follow-up
BR:
34.4% (2007–2011) [49]
21.5% (2010–2019) [63]

Overall

CU: 4.5% (1986–1990), 0.5%(1991–1996) [48]
PE: 12.1% (1989–1993) [54]		At baseline
BR:
30% (2002–2005) [53]
38% (2007–2011) [29]
63% (2010–2011) [52]

Reintervention
BR:
11.5% (1994–2005) [56]
12.7% (1996–2005) [62]
23.07% (2007–2011) [29]
8.3% (Surgery), 10% (PBMV) (1987–2010) [41]
10% (2010–2011) [52]
27.9% (First), 14.8% (Second) (2010–2015) [40]
5.6% (2011–2017) [25]

CL: 4.7% (1990–2004) [55]
Heart failureCU: 11.2% (1986–1990), 1.5% (1991–1996) [48]BR:
22.3% (2009) [23]
7.4% (Postop.) (2011–2017) [25]

CL: 5.1% (1990–2004) [55]
Atrial fibrillationBR:
14% (2007–2011) [49]
30% (2010–2019) [63]		BR:
12.5% (1987–2010) [41]
28% (2007–2011) [29]
53.1% (2009) [23]
0% (2011–2017) [25]

CL: 65.6% (Preop.), 63.3% (Postop.) (1990–2004) [55]
Infective endocarditisCU: 0% (1986–1996) [48]
PE: 23% (1989–1993) [54]		BR:
2.8% (1996–2005) [62]
1.9% (2008–2009) [22]
16% (2010–2011) [52]

CL: 1.4% (1990–2004) [55]

MX: 7.1% [65]
StrokeBR:
18% (Baseline), 5.2% (Follow-up) (2010–2019) [63]
12.7% [36]

PE: 1.5% (1989–1993) [54]		BR:
4.2% (1996–2005) [62]
7.5% (2008–2009) [22]
2.7% (2010–2012) [28]
10.5% (Baseline), 1% (Postop.) (2013–2014) [24]

CL: 2.8% (1990–2004) [55]
Embolic eventsBR: 4.4% [36]BR: 16.4% (Baseline), 12.7% (Postop.) (1991–1994) [35]
MX: 7.1% [65]
Pulmonary hypertensionPE: 16.7% (1989–1993) [54]BR:57.5% (2009) [23]
77.6% (Preop.), 18.4% (Postop.) (2011–2017) [25]

[i] * Supplementary Table S9 includes the information on burden of RHD per each included study.

1 Several studies (‘Intervention-only studies’) that assessed solely surgical or percutaneously intervened RHD patients while others (‘General studies’) assessed patients receiving any kind of treatment.

2 ‘Need for intervention’ includes any surgical (initial or reoperation) or percutaneous intervention (initial or reintervention).

Abbreviations: BR: Brazil, CL: Chile, CU: Cuba, MX: Mexico, PBMV: Percutaneous Balloon Mitral Valvuloplasty; PE: Peru, Postop.: postoperative, Preop.: preoperative, VE: Venezuela.

Table 4

Preventive and screening strategies for rheumatic heart disease in Latin America and the Caribbean.

REFERENCECOUNTRYSTUDY PERIODDESCRIPTIONRESULTS
Prevention programs
Mota et al., 2015 [44]Brazil1977–2000Prevention Program for ARF-UFMG (since 1988)

  1. HCW education.

  2. Outpatient clinic for ARF with routine follow-up.

  3. Echocardiographic screening for definite or suspected cases of ARF or RHD.

  4. Distribution of free medication.

  5. Chemoprophylaxis and ‘prophylaxis card’ to control compliance.

  6. Active searching of missing patients.

Comparing two periods (July 1977–July 1988, n = 248 and August 1988–February 2000, n = 454), the authors identified a decrease in:

  1. Recurrences (22.4 vs 7.4, p = 0.0000)

  2. Hospital admissions (45.4 vs 28.4, p = 0.0000)

  3. Surgeries (13.3 vs 1.5, p = 0.0000)

  4. Deaths (5.4 vs 0.4, p = 0.0000)

Berrios et al., 1993 [21]Chile1982–1988ARF Control and Prevention Program of Southeast Health District (Catholic University Medical School, Santiago, Chile)

  1. Chemoprophylaxis: benzathine penicillin G every 28 days (nonpenicillin allergic) or oral sulfadiazine daily (penicillin allergic)

    • Duration: no carditis: 5 years or until age 18; carditis: 10 years or until age 25; aortic involvement, mitral stenosis or multivalvular: for life.

  2. Follow-up after cessation: regular visits every 3 months, annual examination by cardiologist.

  1. 59 post prophylactic patients (1032 scheduled visits and 3346 patient- months)

  2. Recurrence rate of 0.72 (CI, 0.2 to 2.6) per 100 patients-years of prospective surveillance.

Nordet et al., 2008 [48]Cuba1986–2001Pinar del Rio Project

  1. Primary prevention of ARF/RHD: diagnosis and treatment of GAS pharyngitis.

  2. Secondary prevention of ARF/RHD: case finding, referral, permanent register, surveillance, and chemoprophylaxis for ARF/RHD.

  3. Educational program and personnel training.

  4. Two cross-sectional studies for ARF/RHD prevalence in schoolchildren (5–15 year) were conducted in 1985 and 1996.

  1. Decline in the occurrence and severity of RF/RHD. (See Results section, Tables 1 and 3)

  2. Decline of ARF/RHD incidence in schoolchildren and 5–25-year-olds. (See Results section, Table 1)

  3. Increase in secondary prophylaxis compliance: 1986 (50% regular, 36.5% irregular and 13.5% non-compliance) to 1996 (93.8% regular, 6.2% irregular).

  4. Decline in estimated direct costs of RF/RHD: 145519 USD per year (1986–1990) to 49376 USD per year (1991–1996).

Millard-Bullock, 2012 [42]Jamaica1975–1985, 1989–1995The ARF and RHD Control Program – Jamaica

  1. Primary prevention of ARF (identification and treatment of GAS infections).

  2. Secondary prevention with 4-weekly injections of benzathine penicillin.

  3. Case-finding, registration, and surveillance of patients.

  4. Health education to patients, families, and the public. (Conferences, seminars, posters, etc.)

  1. ARF cases surveys (See Results section, Table 2)

  2. ARF/RHD’s hospitalization cost (1989–1995): J$17 million per year for 3 hospitals.

Bach et al., 1996 [19]Martinique Guadeloupe1982–1992Martinique/Guadeloupe eradication program

  1. Registry of all cases.

  2. Educational program (pamphlets, posters, films, etc.) on sore throat, ARF, and HCW education.

  3. Research (immunological, bacteriological, or genetic studies) on patients with ARF.

  1. Decline of ARF cases in both islands. (78% reduction in Martinique and 74% in Guadeloupe)

  2. Decline of patients requiring open heart surgery due to rheumatic fever carditis before age 18.

  3. Cost reduction of recent childhood rheumatic fever from USD 1,426,000 to USD 100,000 (86% decrease)

WHO Cardiovascular Diseases Unit, 1992 [64]BoliviaEl SalvadorJamaica
(‘Americas’)		1986–1990WHO program for the prevention of ARF/RHD in 16 developing countries
The Americas region was one of the five regions assessed and included Bolivia, El Salvador, and Jamaica. The program included:

  1. Case finding via screening surveys of schoolchildren, hospital retrospective case surveys, and continuing detection/referral of any ARF/RHD confirmed or suspected case from health centers.

  2. Central register of all confirmed ARF/RHD patients.

  3. Follow-up consultation and secondary prophylaxis in local centers.

  4. Personnel (schoolteachers and HCW) training and health education.

The Americas region results:

  1. Five surveys (n: 23,328 schoolchildren) conducted for ARF/RHD prevalence (See Results section, Table 1)

  2. Case detection and registration; a total of 9,645 on the register (35 detected in screening, 881 from other sources and 8,729 known cases)

  3. Rate of coverage of prophylaxis: 47.2% (23.8–75.6%) per 100 patients registered for secondary prophylaxis per month. El Salvador had one of the lowest rates among all 16 countries: 23.8%.

  4. Training of personnel: 2,080 (123 doctors, 1,147 other HCW, and 819 schoolteachers)

  5. Activities for health education: pamphlets and brochures: 24,304, posters: 28, radio/TV programs: 22, and group sessions: 93.

Screening programs
Beaton et al., 2016 [20]
Nascimento et al., 2018 [46]		Brazil2014–2016PROVAR: Rheumatic Valve Disease Screening Program

  1. RHD echocardiographic screening program in Minas Gerais, Brazil.

    • Image acquisition by nonexperts on portable and/or handheld devices.

    • Telemedicine interpretation by experts in Brazil and USA (WHF 2012 criteria).

    • Patients with confirmed abnormalities referred for clinical and echocardiographic follow-up.

  2. RHD educational curriculum delivered in schools and in primary care (See Oliveira et al., 2020 below).

  3. Echocardiographic education of non-experts (n: 6): self-directed educational experience followed by field-testing of school-based handheld echocardiography screening [20].

  1. Screening in 52 public schools, 2 private schools and 3 primary care centers. (See Results Section, Table 1)

  2. Educational curriculum delivered to 29,695 children.

  3. Non-experts’ interpretation of echocardiography: sensitivity 83% and specificity 85% for detecting RHD (borderline or definite) [20].

Nascimento et al., 2021 [45]Brazil2018–2019PROVAR+: Programa de RastreamentO da VAlvopatia Reumatica e outras Doenças Cardiovasculares
A continuation of PROVAR [46]. This publication involved an echocardiographic screening program among pregnant women in prenatal care:

  1. Image acquisition by nonexperts on hand-held devices.

  2. Telemedicine image interpretation by experts in Brazil and USA.

  3. HCW received educational curriculum on echocardiography.

  4. Standard echocardiogram was scheduled for those with significant abnormalities during screening.

  1. 1112 pregnant women were screened. (See Results section, Table 1)

  2. Authors concluded that integrating this type of strategies is possible in the Brazilian system.

Spitzer et al., 2015 [60]Peru2014Echocardiopraphic Screening Program on Schoolchildren at Arequipa, Peru

  1. RHD echocardiographic screening program among schoolchidren (5–16 years) in Arequipa, Peru.

    • Cardiac auscultation.

    • Portable echocardiography by cardiologist.

    • If pathologic findings, a detailed echo by local cardiologist was offered.

    • WHO and WHF classifications were evaluated by 5 cardiologists from Bern University Hospital.

  2. Secondary prophylaxis and regular follow-up for children with borderline/definite (WHF) or probable/definite (WHO) RHD.

  1. 1023 children were screened and pathological findings on echocardiography were reported in 59 children (5.8%) and 45 underwent confirmatory echocardiogram. (See Results section, Table 1)

  2. 21 children (4 with concomitant RHD) had congenital heart disease.

  3. Secondary prophylaxis in six children with WHO definite/probable. RHD, and one with WHF borderline RHD.

Educational programs/interventions
Oliveira et al., 2020 [50]Brazil2016–2017RHD educational strategy by PROVAR researchers
Two educational strategies were assessed by a cluster randomized trial among schoolchildren:

  1. ‘Conventional’: classes with slide presentations provided by a research nurse.

  2. ‘Experimental’: individual interactive modules provided in tablets.

  1. Baseline knowledge of ARF/RHD was low.

  2. Improvement in knowledge was similar immediately after intervention.

  3. After 3 months, worsening in knowledge was observed (similar in both groups).

  4. Authors concluded that these educational strategies improve knowledge (which may be important in prevention). However, retention of knowledge was low.

[i] Abbreviations: ARF: Acute rheumatic fever; GAS: Group A Streptococcus; HCW: Healthcare workers; J$: Jamaican Dollars; UFMG: Federal University of Minas Gerais; PROVAR: Rheumatic Valve Disease Screening Program; PROVAR+: Programa de RastreamentO da VAlvopatia Reumática e outras Doenças Cardiovasculares; RHD: Rheumatic Heart Disease; TV: Television; USD: United States Dollars; WHF: World Heart Federation; WHO: World Health Organization.

DOI: https://doi.org/10.5334/gh.1152 | Journal eISSN: 2211-8179
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Language: English
Submitted on: May 3, 2022
Accepted on: Aug 6, 2022
Published on: Sep 2, 2022
Published by: Ubiquity Press
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year
Keywords:
rheumatic heart disease,
rheumatic fever,
epidemiology,
Latin America,
Caribbean region

© 2022 Maria Alejandra Jaimes-Reyes, Manuel Urina-Jassir, Manuel Urina-Triana, Miguel Urina-Triana, published by Ubiquity Press
This work is licensed under the Creative Commons Attribution 4.0 License.

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