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Survival of Rheumatic Heart Disease in Indonesian Children Cover

Survival of Rheumatic Heart Disease in Indonesian Children

Open Access
|Oct 2022

Figures & Tables

Table 1

2012 WHF criteria for echocardiographic diagnosis of RHD for individuals aged ≤ 20 years.

Definite RHD (either A, B, C, or D)
  1. Pathological MR and at least two morphologicalfeatures of RHD of the MV

  2. MS mean gradient ≥4mmHg*

  3. Pathological AR and at least two morphological features ofRHD of the AV

  4. Borderline disease of both the AVand MV§

Borderline RHD (either A, B, or C)
  1. At least two morphological features of RHD of the MV without pathological MR or MS

  2. Pathological MR

  3. Pathological AR

Normal echocardiographic findings (all of A, B, C, and D)
  1. MR that does not meet all four Doppler echocardiographic criteria (physiological MR)

  2. AR that does not meet all four Doppler echocardiographic criteria (physiological AR)

  3. An isolated morphological feature of RHD of the MV (for example, valvular thickening) without any associated pathological stenosis or regurgitation

  4. D. Morphological feature of RHD of the AV (for example, valvular thickening) without any associated pathological stenosis or regurgitation

[i] *Congenital MV anomalies must be excluded. Furthermore, inflow obstruction due to nonrheumatic mitral annular calcification must be excluded in adults. Bicuspid AV, dilated aortic root, and hypertension must be excluded. §Combined AR and MR in high prevalence regions and in the absence of congenital heart disease is regarded as rheumatic. Abbreviations: AR, aortic regurgitation; AV, aortic valve; MR, mitral regurgitation; MS, mitral stenosis; MV, mitral valve; RHD, rheumatic heart disease; WHF, World Heart Federation.

Table 2

Valve severity based on American College of Cardiology/American Heart Association recommendations.

ECHOCARDIOGRAPHIC PARAMETERSMITRAL REGURGITATION
MILDMODERATESEVERE
Color Doppler jet areaSmall central jet area <20% LA1 areaCentral jet MR 20%–40% LA or late systolic eccentric jet MRCentral jet MR2 > 40% LA or holosystolic eccentric jet MR
Vena contracta width (cm)Small vena contracta <0.3 cm0.3–0.6 cm≥0.7 cm
Regurgitant volumeLess than 30 ml30–59 mL≥60 mL
Regurgitant fractionLess than 30%<50%≥50%
AORTIC REGURGITATION
MILDMODERATESEVERE
Jet width<25% of LVOT325%-64% of LVOT≥65% of LVOT
Vena contracta<0.3 cm0.3–0.6 cm≥0.7 cm
Regurgitant volumeLess than 30 ml30–59 mL≥60 mL
Regurgitant fractionLess than 30%<50%≥50%

[i] 1LA, Left atrium.

2MR, Mitral regurgitation.

3LVOT, Left ventricular outflow tract.

Table 3

Baseline characteristics of 155 children with RHD.

CHARACTERISTICSSUBJECTS (n = 155)
Age in years, mean (SD)11.9 (2.9)
Sex, n (%)
  • – Male

90 (58.1)
  • – Female

65 (41.9)
Complications, n (%)
  • – Infective endocarditis

2 (1.3)
  • – Atrial fibrillation

4 (2.6)
  • – Congestive heart failure

27 (17.4)
  • – Pericardial effusion

11 (7.1)
  • – Pneumonia

2 (1,3)
  • Total AV Block

1 (0.6)
  • – Pulmonary hypertension

38 (24.5)
NYHA functional class at initial presentation, n (%)
  • – NYHA I

29 (18.7)
  • – NYHA II

40 (25.8)
  • – NYHA III

64 (41.2)
  • – NYHA IV

22 (14.2)
Echocardiography parameter:
  • – LVIDd, median (min-max)

51.2 (32.5–85.0)
  • – EF (%), median (min-max)

64.4 (15.0–82.0)
  • – Valve lesion severity, n (%)

               Severe96 (61.1)
               Moderate34 (22.1)
               Mild25 (16.8)

[i] AV, atrioventricular.

LVIDd, Left Ventricular Internal Diameter end distole;

EF, Ejection Fraction.

NYHA, New York Heart Association.

Table 4

Cause of deaths and comorbidities of patients with RHD.

CAUSE OF DEATHn (%)
Intracranial hemorrhage1 (0.6)
Cardiogenic shock12 (7.7)
Pulmonary hypertension crisis1 (0.6)
COMORBIDITYn (%)
Endocarditis1 (0.6)
Thyroid malignancy1 (0.6)
Pneumonia4 (2.4)
Scabies1 (0.6)
Total atrioventricular block1 (0.6)
Figure 1

Study flow chart and follow up.

Table 5

Characteristics of valves lesions in children with RHD (n = 155).

VALVES LESIONSn (%)
a. Single valve lesion
Mitral valve:
  • – Mitral regurgitation (MR)a

41 (27.5)
  • – Mitral stenosis (MS)b

0 (0.0)
Aortic valve
  • – Aortic regurgitation (AR)c

3 (2.0)
  • – Aortic stenosis (AS)d

0 (0.0)
  • – Combination of ARc and ASd

0 (0.0)
b. Multiple valve lesions
  • – MRa + ARc

41 (26.5)
  • – MRa + Tricuspid regurgitation (TR)e

20 (12.9)
  • – MRa + Pulmonal regurgitationf

2 (1.4)
  • – MRa + MSb +TRe

1 (0.7)
  • – MRa + MSb + ARc + TRe

3 (2.0)
  • – MRa + ARc + TRe

21 (13.5)
  • – MRa + TRe + PRf

8 (5.4)
  • – MRa + ARc + PRf

1 (0.7)
  • – MRa + ARc + TRe + PRf

14 (9.0)

[i] Notes:

aMR, Mitral Regurgitation; bMS, Mitral Stenosis; cAR, Aortic Regurgitation; dAS, Aortic Stenosis; eTR, Tricuspid Regurgitation; fPR, Pulmonal Regurgitation.

Figure 2

Survival analysis of children with RHD.

Figure 3

Kaplan-Meier curve stratified by valve severity.

Figure 4

Kaplan-Meier curve stratified by NYHA functional class at first diagnosis.

Table 6

Predictors of mortality in children with RHD with Cox Proportional Hazard Model.

PREDICTORSDIED (n = 14)ALIVE (n = 141)UNADJUSTED HAZARD RATIO (CI 95%)pADJUSTED HAZARD RATIO (CI 95%)p
Age in years, mean (SD)12.8 (2.8)11.8 (2.9)1.2 (0.9–1.5)0.06
Severe malnutrition, n (%)8 (57.1)13 (9.2)7.3 (2.5–21.5)<0.00011.6 (0.2–14.1)0.95
Atrial fibrillation, n (%)3 (21.4)1 (0.7)1.4 (0.8–22.3)0.07
Congestive heart failure, n (%)10 (71.4)16 (11.3)11.5 (3.6–36.7)<0.00018.9 (0.7–118.8)0.09
Pulmonary hypertension, n (%)11 (78.6)28 (19.9)10.1 (2.8–36.3)<0.00010.9 (0.1–9.7)0.98
Poor adherence to penicillin prophylaxis8 (88.9)40 (28.6)34.6 (4.3–282.8)<0.000114.3 (1.3–156.6)0.02
EF < 55%, n (%)4 (28.6)16 (11.3)2.9 (0.8–9.4)0.07
Z-scores LVIDd, mean (SD)3.2 (2.1)1.6 (1.9)1.5 (1.1–2.1)0.021.1 (0.6–2.3)0.71

[i] Severe malnutrition, Body mass index-per-age ≤ –3SD based on WHO 2006 growth chart.

‘Non-adherent’ or ‘poor adherent’ referred to the consumptions of oral penicillin were less than 80% of described doses based on patients’ self-reports for 12 months.

LVIDd, Left Ventricular Internal Diameter end distole body surface area-adjusted z-scores

EF, Ejection Fraction

Table 7

Simplified multivariable Cox Proportional Hazard Model of RHD mortality predictors.

PREDICTORSADJUSTED HAZARD RATIO (CI 95%)p
Severe malnutrition, n (%)0.6 (0.1–3.2)0.51
Congestive heart failure, n (%)18.6 (1.8–185.0)0.01
Poor adherence to penicillin prophylaxis12.7 (1.4–113.5)0.02
DOI: https://doi.org/10.5334/gh.1160 | Journal eISSN: 2211-8179
Language: English
Submitted on: May 16, 2022
Accepted on: Sep 9, 2022
Published on: Oct 6, 2022
Published by: Ubiquity Press
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year

© 2022 Nadya Arafuri, Indah Kartika Murni, Madarina Julia, Sasmito Nugroho, Noormanto Soehadi, published by Ubiquity Press
This work is licensed under the Creative Commons Attribution 4.0 License.