Table 1
Common RHD valve lesions and associated auscultation findings.
| VALVE LESION | AUSCULTATION FINDINGS |
|---|---|
| Mitral regurgitation | Mid/pan-systolic murmur at apex, radiating laterally (occasionally medially/ posteriorly) |
| Mitral stenosis | Low-pitch, diastolic murmur at apex with patient in left lateral position. |
| Aortic regurgitation | Blowing decrescendo diastolic murmur at left sternal edge. Systolic murmur due to increased flow Mitral diastolic murmur (Austin Flint) |
| Aortic stenosis | Ejection systolic murmur over aortic region, radiating to the neck. |
Table 2
The WHF 2012 criteria for the echocardiographic diagnosis of RHD.
| A) 2012 WHF guidelines for diagnosis of RHD |
| Definite RHD (either A, B, C or D): A. Pathological MR and at least two morphological features of RHD of the MV B. MS mean gradient ≥4 mmHg C. Pathological AR and at least two morphological features of RHD of the AV D. Borderline disease of both the AV and MV Borderline RHD (either A, B, or C) A. At least two morphological features of RHD of the MV without pathological MR or MS B. Pathological MR C. Pathological AR |
| B) Criteria for pathological regurgitation |
Pathological mitral regurgitation (All four Doppler echocardiographic criteria must be met)
Pathological aortic regurgitation (All four Doppler echocardiographic criteria must be met)
|
| C) Morphological features of RHD |
Features in the MV
Features in the AV
|
[i] WHF: World Heart Federation; RHD: Rheumatic Heart Disease; MR: Mitral Regurgitation; MV: Mitral Valve; AR: Aortic Regurgitation; AV: Aortic Valve; MS: Mitral Stenosis; AMVL: Anterior Mitral Valve Leaflet
Table 3
Summary of the new features of the 2023 WHF guidelines [7].
|
Figure 1
Flow diagram for literature search for studies related to performance of the 2012 WHF criteria for the echocardiographic diagnosis of RHD.
Table 4
Studies of inter-reviewer reliability of the 2012 WHF criteria.
| Diagnostic Feature(s) | Kane A et al. [27] | Bacquelin R et al. [13] | Culliford-Semmens et al. [14] | Remenyi et al. [15] | Beaton A et al. [16] | Scheel A et al. [12] |
|---|---|---|---|---|---|---|
| Criteria as a whole | Concordance between 3 reviewers; (6–15yo) 60%, 40%, 20%; (16–18) 100%, 90%, 90%. | k = 0.83 (95% CI 0.74–0.93) * | k = 0.57 (CI 0.44–0.7) ^2 reviewer consensus 60%3 reviewer consensus 89% | No/bor/def RHD; k = 0.49 (95% CI 0.45–0.54), def RHD; k = 0.65 (95% CI 0.59–0.7)any RHD; k = 0.6 (95% CI 0.55–0.65) | k = 1.0 | Any RHD; k = 0.66 (95% CI 0.61–0.71),no/bor/def RHD; k = 0.51,def RHD: k = 0.76 |
| Pathological Mitral Regurgitation | — | k = 0.92 (95% CI 0.83–1.0) | — | k = 0.60 (95% CI 0.55–0.65) | k = 1.0 | k = 0.69 |
| Morphological Features of Mitral Valve | — | k = 0.83 (95% CI 0.72–0.94) | — | k = 0.57 (95% CI 0.5 –0.62) | — | k = 0.57 |
| Mitral Regurgitation Jet Length | — | — | — | — | ICC = 0.90 | — |
| AMVL Thickness | — | k = 0.6 (95% CI 0.43–0.77) | — | k = 0.75 (95% CI 0.7–0.8) | ICC = 0.57 | k = 0.6 |
| Restricted Motion | — | k = 0.53 (95% CI 0.35–0.7) | — | k = 0.55 (95% CI 0.49–0.6) | k = 0.69 | k = 0.7 |
| Chordal Thickening | — | k = 0.62 (95% CI 0.46–0.78) | — | k = 0.54 (95% CI 0.49–0.59) | k = 0.41 | k = 0.78 |
| Excessive Leaflet Tip Motion | — | — | — | k = 0.63 (95% CI 0.58–0.68) | too low to assess | k = 0.74 |
| Pathological Aortic Regurgitation | — | — | — | k = 0.86 | — | k = 0.95 |
| Morphological features of Aortic Valve | — | — | — | — | too low to assess | k = 0.89 |
[i] AMVL: Anterior mitral valve leaflet, bor: Borderline RHD, def: Definite RHD k: Kappa, ICC: Intraclass correlation coefficient.
Table 5
Studies of abbreviation of the WHF 2012 criteria and their accuracy when performed by an expert.
| Diagnostic Feature(s) | Diamantino et al. [29] | Beaton et al. [21] | Lu et al. [28] | Remenyi et al. [30] | Zuhlke et al. [24] |
|---|---|---|---|---|---|
| Modified protocol | PLAX with colour Doppler. | 11 image protocol (same as STAND) | 11 image protocol, excluded CW | PLAX with sweep of the heart and color Doppler | PLAX with color Doppler |
| Modified criteria | Valve regurgitation only | CW replaced with regurgitation jet seen in ≥2 consecutive frames | Assessed multiple parameters that maximised sensitivity and specificity | Valve regurgitation or morphological features | |
| Definition of screen positive | MR ≥1.5 cm, any AR | N/A | MR ≥1.5 cm or any AR found to have the best compromise | Any MR or AR seen in 2 consecutive frames, ≥2 morph features of MV | MR ≥2 cm |
| Diagnosis of RHD | As per WHF | As per WHF | As per WHF | As per WHF | As per WHF |
| Sensitivity c/f ref WHF criteria by standard echocardiography for any RHD | 81.10% | 78.9%% | 73.30% | 100% | 80.80% |
| Specificity c/f ref WHF criteria by standard echocardiography for any RHD | 75.50% | 87.2%% | 82.40% | 95% | 100% |
| PPV & NPV for any RHD | 63.2%; 88.5% | 44%; 100% | 100%, 92.5% |
Table 6
Sensitivity and specificity of abbreviated 2012 WHF criteria when performed by non-experts.
| Engelman et al. [17] | Francis et al. [18] | Francis et al. [31] | Mirabel et al. [25] | Ploutz et al. [23] | |
|---|---|---|---|---|---|
| Non-experts | Nurses | Nurses, Indigenous health workers, physicians | Nurses, Indigenous health workers, physicians | Nurses | Nurses |
| Modified protocol and criteria | 12-step protocol. | Single PLAX with a sweep through the heart. | Single PLAX with a sweep through the heart. | PLAX, PSAX, A2C, A3C, A4C | PLAX, A4C, A5C |
| Any MR or AR | Any MR or AR | Any MR or AR Approach 1: non-expert screen and refer. Approach 2 non-expert screen with expert off-sight review to determine referral | MR ≥2 cm or any AR | MR ≥1.5 cm and/or any AR | |
| Gold standard | Comprehensive echo performed by Cardiologist applying 2012 WHF criteria | Comprehensive echo performed by Cardiologist applying 2012 WHF criteria | Abbreviated echo performed by expert & Comprehensive echo performed by Cardiologist applying 2012 WHF criteria | Comprehensive echo reviewed by Cardiologist applying 2012 WHF criteria | Comprehensive echo performed by Cardiologist applying 2012 WHF criteria |
| Findings | Sensitivity: 84.2% (95% CI 72.1–92.5) | Sensitivity: 70.4% | Approach 1: Sensitivity: 86.5%, Specificity: 61.4% | Sensitivity: 77.6%–83.7% | Sensitivity: 74.4% (95% CI 58.8–86.5) |
| Specificity: 85.6% (95% CI 83.9–87.1%) | Specificity: 78.1% | Approach 2: Sensitivity: 88.4%, Specificity: 77.1% | Specificity: 90.9%–92% (variation differing between operators) | Specificity: 78.8% (95% CI 76.0–81.4) |
Table 7
Simplified score as per Nunes et al. [35]) and associated points conveying risk of disease progression (low risk: 1–6, intermediate risk: 6–10, high risk >10).
| VARIABLE | POINTS |
|---|---|
| Mitral valve | |
| Anterior leaflet thickening | 3 |
| Excessive leaflet tip motion | 3 |
| Regurgitation jet length ≥2 cm | 6 |
| Aortic valve | |
| Irregular or focal thickening | 4 |
| Any regurgitation | 5 |