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The Impact of Acute Rheumatic Fever Diagnosis on Rheumatic Heart Disease Severity Cover

The Impact of Acute Rheumatic Fever Diagnosis on Rheumatic Heart Disease Severity

Open Access
|Aug 2025

Figures & Tables

Table 1

Rheumatic heart disease stages (9).

Stage A
  • mild isolated pathological MR, or

  • mild isolated pathological AR.

Stage B
  • mild pathological MR and mild pathological AR, or

  • mild pathological MR with morphological abnormality/s* of the mitral valve, or

  • mild pathological AR with morphological abnormality/s* of the aortic valve.

Stage C
  • at least moderate MR, or

  • at least moderate AR, or

  • any mitral stenosis, or

  • any aortic stenosis.

Stage D
  • any of the Stage C lesions requiring surgical intervention**

[i] *At least one morphological abnormality required if ≤ 20 years; at least two abnormalities required if >20 years.

**The guidelines specify the presence of clinical complications including the need for surgical intervention constitutes Stage D RHD. In the Australian setting, surgery is an option for all those requiring it, and since this audit did not include information on clinical history, surgical events provided by the RHD register were used to indicate the presence of Stage D RHD in this cohort.

Figure 1

Patient inclusion and stratification process. Registry data identified individuals with RHD and ARF diagnoses. Echocardiographic images were reviewed to determine RHD stage at baseline and at follow-up.

Table 2

Summary of patient data, RHD severity, and lesion type at baseline.

ARF n = 192 n (%)*No ARF n = 100 n (%)*P-value
Age at baseline (years; mean (sd))11.4 (3.7)12.8 (3.9)0.002
Female96 (50.0%)65 (65.0%)0.018
RHD stages0.009
Early RHD – Stage A55 (28.6%)12 (12.0%)
Early RHD – Stage B73 (38.0%)50 (50.0%)
Advanced RHD – Stage C55 (28.6%)31 (31.0%)
Advanced RHD – Stage D9 (4.7%)7 (7.0%)
Total early RHD128 (66.7%)62 (62.0%)0.440
Total advanced RHD64 (33.3%)38 (38.0%)
Lesion type
Pathological mitral regurgitation – all**169 (88.0%)88 (88.0%)0.996
Pathological aortic regurgitation – all**85 (44.3%)39 (39%)0.387
Isolated mitral regurgitation40 (20.8%)7 (7.0%)0.002
Isolated aortic regurgitation17 (8.9%)6 (6.0%)0.495
MR and AR (no abnormal morphology)10 (5.2%)3 (3.0%)0.553
MR with abnormal morphology (+/– AV disease)117 (60.9%)76 (76.0%)0.010
AR with abnormal morphology (+/– MV disease)33 (17.2%)20 (20.0%)0.554
Any mitral stenosis4 (2.1%)3 (3.0%)0.694
Any aortic stenosis0 (0%)1 (1.0%)0.343

[i] *Column percentage, unless otherwise specified. Fisher test, unless otherwise specified.

**Includes pathological regurgitation in isolation and in combination with other valve lesions.

AV, aortic valve; ARF, acute rheumatic fever; AR, aortic regurgitation; MR, mitral regurgitation; MV, mitral valve; RHD, rheumatic heart disease, sd, standard deviation.

Figure 2

RHD stage at baseline echocardiogram stratified by the presence of ARF or no ARF.

Table 3

Summary of patient data RHD severity and lesion type at follow-up.

ARF n = 156 n (%)*No ARF n = 74 n (%)*P-value
Age at follow-up (years; mean (sd))*15.4 (4.2)16.8 (4.9)0.031
Female78 (50%)50 (67.6%)0.016
SAP adherence % (median, IQR) n = 19781 (66–88)82 (69–89)0.966
RHD stages0.008
Resolved RHD45 (28.8%)13 (17.6%)
Early RHD – Stage A20 (12.8%)5 (7.1%)
Early RHD – Stage B53 (34.0%)33 (44.6%)
Advanced RHD – Stage C25 (16.0%)17 (23.0%)
Advanced RHD – Stage D13 (8.3%)6 (8.1%)
Total early RHD73 (46.8%)38 (51.4%)0.165
Total advanced RHD38 (24.4%)23 (31.1%)
Resolved RHD45 (28.8%)13 (17.6%)
Lesion type (resolved RHD excluded)n = 111n = 61
Pathological mitral regurgitation – all92 (82.9%)56 (91.8%)0.166
Pathological aortic regurgitation – all43 (38.7%)16 (26.2%)0.131
Isolated mitral regurgitation11 (9.9%)2 (3.3%)0.141
Isolated aortic regurgitation9 (8.1%)3 (4.9%)0.542
MR and AR (no abnormal morphology)0 (0%)1 (1.6%)0.355
MR with abnormal morphology (+/– AV disease)80 (72.1%)53 (86.9%)0.036
AR with abnormal morphology (+/– MV disease)26 (23.4%)10 (16.4%)0.330
Any mitral stenosis9 (8.1%)3 (4.9%)0.542
Any aortic stenosis0 (0%)0 (0%)

[i] *Column percentage, unless otherwise specified. Fisher test, unless otherwise specified.

AV, aortic valve; ARF, acute rheumatic fever; AR, aortic regurgitation; MR, mitral regurgitation; RHD, rheumatic heart disease, SAP, secondary antibiotic prophylaxis; sd, standard deviation.

Figure 3

RHD stages at follow-up stratified by ARF and noARF status at baseline.

Figure 4

Sankey diagram demonstrating changes in RHD stage between baseline and follow-up echocardiogram. 4A shows those with ARF at baseline. 4B shows those with noARF at baseline. The width of each ribbon demonstrates the relative proportion of individuals in each stage at baseline and at follow-up.

Figure 5

Progression/regression of RHD stage from baseline to end of follow-up period stratified by ARF status.

DOI: https://doi.org/10.5334/gh.1454 | Journal eISSN: 2211-8179
Language: English
Submitted on: Feb 1, 2025
Accepted on: Jul 23, 2025
Published on: Aug 29, 2025
Published by: Ubiquity Press
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year

© 2025 Jacqueline Maree Williamson, Gillian Whalley, Simon Thornley, James Marangou, Peter Morris, Joshua R. Francis, Vicki Wade, Bo Remenyi, published by Ubiquity Press
This work is licensed under the Creative Commons Attribution 4.0 License.