| Variables . | Pooled estimate . | Range . | Studies (n) . |
|---|---|---|---|
| Patient characteristics | |||
| Age (years), mean (SD) | 4.68 (5.84) | 2.2–14.3 | 23 |
| Male (%) | 62.1 | 44.9–72.7 | 22 |
| Weight (kg), mean (SD) | 20.7 (17.1) | 11.5–36.9 | 14 |
| Discrete type SVAS (%) | 71.7 | 14.3–100 | 20 |
| Diffuse type SVAS (%) | 28.0 | 0.0–85.7 | 20 |
| Williams–Beuren syndrome (%) | 49.6 | 13.9–100 | 22 |
| Sporadic SVAS (%) | 30.9 | 0.0–58.8 | 7 |
| NYHA class >I (%) | 62.3 | 9.9–89.1 | 5 |
| Symptomatic (%) | 44.0 | 23.0–91.0 | 5 |
| Associated cardiovascular anomalies | |||
| Concomitant anomalies (%)a | 80.4 | 13.0–100 | 19 |
| Branch and/or peripheral PA stenosis (%) | 20.1 | 0.0–65.0 | 21 |
| AV stenosis (%) | 17.0 | 0.0–34.7 | 15 |
| Aortic anomalies (%) | 13.0 | 0.0–100 | 16 |
| Aortic coarctation (%) | 6.38 | 0.0–26.5 | 18 |
| Aortic arch abnormality (%) | 2.33 | 0.0–10.7 | 16 |
| BAV (%) | 11.1 | 0.0–38.9 | 18 |
| AV regurgitation (%) | 6.44 | 0.0–30.6 | 15 |
| Coronary anomaly (%) | 10.8 | 0.0–28.0 | 17 |
| Coronary stenosis (%) | 6.89 | 0.0–36.0 | 19 |
| SAS (%) | 7.99 | 0.0–30.6 | 16 |
| (Sub)pulmonary valve stenosis (%) | 3.84 | 0.0–22.5 | 18 |
| VSD (%) | 2.65 | 0.0–10.9 | 17 |
| Previous cardiac interventions | |||
| Previous interventions (%)a | 24.3 | 3.6–47.5 | 10 |
| Aortic surgery (%) | 6.98 | 0.0–16.0 | 9 |
| Coarctectomy (%) | 5.95 | 0.0–16.0 | 11 |
| Aortic valvuloplasty/valve repair (%) | 3.12 | 0.0–11.1 | 9 |
| SAS resection (%) | 1.19 | 0.0–8.3 | 9 |
| SVAS resection (%) | 0.74 | 0.0–8.3 | 9 |
| VSD closure (%) | 0.74 | 0.0–4.0 | 9 |
| Other/unknown | 9.59 | 0.0–25.5 | 11 |
| Operative characteristics SVAS repair | |||
| Type of SVAS repair | |||
| McGoon repair/single-patch repair (%) | 46.3 | 0.0–100 | 23 |
| Doty repair/Y-shaped/pantaloons shaped patch (%) | 33.2 | 0.0–100 | 23 |
| Brom repair/three-patch repair (%) | 14.2 | 0.0–100 | 23 |
| Myers sliding aortoplasty (%) | 2.63 | 0.0–52.0 | 23 |
| Other/unknown (%) | 3.66 | 0.0–30.6 | 23 |
| Concomitant procedures | |||
| Concomitant procedures excluding SVAS/SAS/AV (%)a | 31.4 | 0.0–107.9 | 22 |
| Pulmonary and other procedures (%) | 24.5 | 3.85–73.0 | 22 |
| Aortic surgery (%) | 3.65 | 0.0–20.6 | 22 |
| Other valve repair/replacement (%) | 2.60 | 0.0–16.7 | 22 |
| AV surgery (%) | 10.3 | 0.0–38.9 | 22 |
| SAS resection (%) | 5.61 | 0.0–30.6 | 22 |
| Variables . | Pooled estimate . | Range . | Studies (n) . |
|---|---|---|---|
| Patient characteristics | |||
| Age (years), mean (SD) | 4.68 (5.84) | 2.2–14.3 | 23 |
| Male (%) | 62.1 | 44.9–72.7 | 22 |
| Weight (kg), mean (SD) | 20.7 (17.1) | 11.5–36.9 | 14 |
| Discrete type SVAS (%) | 71.7 | 14.3–100 | 20 |
| Diffuse type SVAS (%) | 28.0 | 0.0–85.7 | 20 |
| Williams–Beuren syndrome (%) | 49.6 | 13.9–100 | 22 |
| Sporadic SVAS (%) | 30.9 | 0.0–58.8 | 7 |
| NYHA class >I (%) | 62.3 | 9.9–89.1 | 5 |
| Symptomatic (%) | 44.0 | 23.0–91.0 | 5 |
| Associated cardiovascular anomalies | |||
| Concomitant anomalies (%)a | 80.4 | 13.0–100 | 19 |
| Branch and/or peripheral PA stenosis (%) | 20.1 | 0.0–65.0 | 21 |
| AV stenosis (%) | 17.0 | 0.0–34.7 | 15 |
| Aortic anomalies (%) | 13.0 | 0.0–100 | 16 |
| Aortic coarctation (%) | 6.38 | 0.0–26.5 | 18 |
| Aortic arch abnormality (%) | 2.33 | 0.0–10.7 | 16 |
| BAV (%) | 11.1 | 0.0–38.9 | 18 |
| AV regurgitation (%) | 6.44 | 0.0–30.6 | 15 |
| Coronary anomaly (%) | 10.8 | 0.0–28.0 | 17 |
| Coronary stenosis (%) | 6.89 | 0.0–36.0 | 19 |
| SAS (%) | 7.99 | 0.0–30.6 | 16 |
| (Sub)pulmonary valve stenosis (%) | 3.84 | 0.0–22.5 | 18 |
| VSD (%) | 2.65 | 0.0–10.9 | 17 |
| Previous cardiac interventions | |||
| Previous interventions (%)a | 24.3 | 3.6–47.5 | 10 |
| Aortic surgery (%) | 6.98 | 0.0–16.0 | 9 |
| Coarctectomy (%) | 5.95 | 0.0–16.0 | 11 |
| Aortic valvuloplasty/valve repair (%) | 3.12 | 0.0–11.1 | 9 |
| SAS resection (%) | 1.19 | 0.0–8.3 | 9 |
| SVAS resection (%) | 0.74 | 0.0–8.3 | 9 |
| VSD closure (%) | 0.74 | 0.0–4.0 | 9 |
| Other/unknown | 9.59 | 0.0–25.5 | 11 |
| Operative characteristics SVAS repair | |||
| Type of SVAS repair | |||
| McGoon repair/single-patch repair (%) | 46.3 | 0.0–100 | 23 |
| Doty repair/Y-shaped/pantaloons shaped patch (%) | 33.2 | 0.0–100 | 23 |
| Brom repair/three-patch repair (%) | 14.2 | 0.0–100 | 23 |
| Myers sliding aortoplasty (%) | 2.63 | 0.0–52.0 | 23 |
| Other/unknown (%) | 3.66 | 0.0–30.6 | 23 |
| Concomitant procedures | |||
| Concomitant procedures excluding SVAS/SAS/AV (%)a | 31.4 | 0.0–107.9 | 22 |
| Pulmonary and other procedures (%) | 24.5 | 3.85–73.0 | 22 |
| Aortic surgery (%) | 3.65 | 0.0–20.6 | 22 |
| Other valve repair/replacement (%) | 2.60 | 0.0–16.7 | 22 |
| AV surgery (%) | 10.3 | 0.0–38.9 | 22 |
| SAS resection (%) | 5.61 | 0.0–30.6 | 22 |
Pooled percentages are depicted for categorical variables and pooled mean/median with standard deviation in parentheses for continuous variables. The range of the mean/median values or percentages in the reporting studies is presented.
The number of concomitant anomalies and interventions are reported; therefore, the percentage can reach higher than 100% as multiple concomitant anomalies of cardiac interventions could be reported per patient.
AV: aortic valve; BAV: bicuspid aortic valve; NYHA: New York Heart Association; PA: pulmonary artery; SAS: subvalvular aortic stenosis; SD: standard deviation; SVAS: supravalvular aortic stenosis; VSD: ventricular septal defect.
| Variables . | Pooled estimate . | Range . | Studies (n) . |
|---|---|---|---|
| Patient characteristics | |||
| Age (years), mean (SD) | 4.68 (5.84) | 2.2–14.3 | 23 |
| Male (%) | 62.1 | 44.9–72.7 | 22 |
| Weight (kg), mean (SD) | 20.7 (17.1) | 11.5–36.9 | 14 |
| Discrete type SVAS (%) | 71.7 | 14.3–100 | 20 |
| Diffuse type SVAS (%) | 28.0 | 0.0–85.7 | 20 |
| Williams–Beuren syndrome (%) | 49.6 | 13.9–100 | 22 |
| Sporadic SVAS (%) | 30.9 | 0.0–58.8 | 7 |
| NYHA class >I (%) | 62.3 | 9.9–89.1 | 5 |
| Symptomatic (%) | 44.0 | 23.0–91.0 | 5 |
| Associated cardiovascular anomalies | |||
| Concomitant anomalies (%)a | 80.4 | 13.0–100 | 19 |
| Branch and/or peripheral PA stenosis (%) | 20.1 | 0.0–65.0 | 21 |
| AV stenosis (%) | 17.0 | 0.0–34.7 | 15 |
| Aortic anomalies (%) | 13.0 | 0.0–100 | 16 |
| Aortic coarctation (%) | 6.38 | 0.0–26.5 | 18 |
| Aortic arch abnormality (%) | 2.33 | 0.0–10.7 | 16 |
| BAV (%) | 11.1 | 0.0–38.9 | 18 |
| AV regurgitation (%) | 6.44 | 0.0–30.6 | 15 |
| Coronary anomaly (%) | 10.8 | 0.0–28.0 | 17 |
| Coronary stenosis (%) | 6.89 | 0.0–36.0 | 19 |
| SAS (%) | 7.99 | 0.0–30.6 | 16 |
| (Sub)pulmonary valve stenosis (%) | 3.84 | 0.0–22.5 | 18 |
| VSD (%) | 2.65 | 0.0–10.9 | 17 |
| Previous cardiac interventions | |||
| Previous interventions (%)a | 24.3 | 3.6–47.5 | 10 |
| Aortic surgery (%) | 6.98 | 0.0–16.0 | 9 |
| Coarctectomy (%) | 5.95 | 0.0–16.0 | 11 |
| Aortic valvuloplasty/valve repair (%) | 3.12 | 0.0–11.1 | 9 |
| SAS resection (%) | 1.19 | 0.0–8.3 | 9 |
| SVAS resection (%) | 0.74 | 0.0–8.3 | 9 |
| VSD closure (%) | 0.74 | 0.0–4.0 | 9 |
| Other/unknown | 9.59 | 0.0–25.5 | 11 |
| Operative characteristics SVAS repair | |||
| Type of SVAS repair | |||
| McGoon repair/single-patch repair (%) | 46.3 | 0.0–100 | 23 |
| Doty repair/Y-shaped/pantaloons shaped patch (%) | 33.2 | 0.0–100 | 23 |
| Brom repair/three-patch repair (%) | 14.2 | 0.0–100 | 23 |
| Myers sliding aortoplasty (%) | 2.63 | 0.0–52.0 | 23 |
| Other/unknown (%) | 3.66 | 0.0–30.6 | 23 |
| Concomitant procedures | |||
| Concomitant procedures excluding SVAS/SAS/AV (%)a | 31.4 | 0.0–107.9 | 22 |
| Pulmonary and other procedures (%) | 24.5 | 3.85–73.0 | 22 |
| Aortic surgery (%) | 3.65 | 0.0–20.6 | 22 |
| Other valve repair/replacement (%) | 2.60 | 0.0–16.7 | 22 |
| AV surgery (%) | 10.3 | 0.0–38.9 | 22 |
| SAS resection (%) | 5.61 | 0.0–30.6 | 22 |
| Variables . | Pooled estimate . | Range . | Studies (n) . |
|---|---|---|---|
| Patient characteristics | |||
| Age (years), mean (SD) | 4.68 (5.84) | 2.2–14.3 | 23 |
| Male (%) | 62.1 | 44.9–72.7 | 22 |
| Weight (kg), mean (SD) | 20.7 (17.1) | 11.5–36.9 | 14 |
| Discrete type SVAS (%) | 71.7 | 14.3–100 | 20 |
| Diffuse type SVAS (%) | 28.0 | 0.0–85.7 | 20 |
| Williams–Beuren syndrome (%) | 49.6 | 13.9–100 | 22 |
| Sporadic SVAS (%) | 30.9 | 0.0–58.8 | 7 |
| NYHA class >I (%) | 62.3 | 9.9–89.1 | 5 |
| Symptomatic (%) | 44.0 | 23.0–91.0 | 5 |
| Associated cardiovascular anomalies | |||
| Concomitant anomalies (%)a | 80.4 | 13.0–100 | 19 |
| Branch and/or peripheral PA stenosis (%) | 20.1 | 0.0–65.0 | 21 |
| AV stenosis (%) | 17.0 | 0.0–34.7 | 15 |
| Aortic anomalies (%) | 13.0 | 0.0–100 | 16 |
| Aortic coarctation (%) | 6.38 | 0.0–26.5 | 18 |
| Aortic arch abnormality (%) | 2.33 | 0.0–10.7 | 16 |
| BAV (%) | 11.1 | 0.0–38.9 | 18 |
| AV regurgitation (%) | 6.44 | 0.0–30.6 | 15 |
| Coronary anomaly (%) | 10.8 | 0.0–28.0 | 17 |
| Coronary stenosis (%) | 6.89 | 0.0–36.0 | 19 |
| SAS (%) | 7.99 | 0.0–30.6 | 16 |
| (Sub)pulmonary valve stenosis (%) | 3.84 | 0.0–22.5 | 18 |
| VSD (%) | 2.65 | 0.0–10.9 | 17 |
| Previous cardiac interventions | |||
| Previous interventions (%)a | 24.3 | 3.6–47.5 | 10 |
| Aortic surgery (%) | 6.98 | 0.0–16.0 | 9 |
| Coarctectomy (%) | 5.95 | 0.0–16.0 | 11 |
| Aortic valvuloplasty/valve repair (%) | 3.12 | 0.0–11.1 | 9 |
| SAS resection (%) | 1.19 | 0.0–8.3 | 9 |
| SVAS resection (%) | 0.74 | 0.0–8.3 | 9 |
| VSD closure (%) | 0.74 | 0.0–4.0 | 9 |
| Other/unknown | 9.59 | 0.0–25.5 | 11 |
| Operative characteristics SVAS repair | |||
| Type of SVAS repair | |||
| McGoon repair/single-patch repair (%) | 46.3 | 0.0–100 | 23 |
| Doty repair/Y-shaped/pantaloons shaped patch (%) | 33.2 | 0.0–100 | 23 |
| Brom repair/three-patch repair (%) | 14.2 | 0.0–100 | 23 |
| Myers sliding aortoplasty (%) | 2.63 | 0.0–52.0 | 23 |
| Other/unknown (%) | 3.66 | 0.0–30.6 | 23 |
| Concomitant procedures | |||
| Concomitant procedures excluding SVAS/SAS/AV (%)a | 31.4 | 0.0–107.9 | 22 |
| Pulmonary and other procedures (%) | 24.5 | 3.85–73.0 | 22 |
| Aortic surgery (%) | 3.65 | 0.0–20.6 | 22 |
| Other valve repair/replacement (%) | 2.60 | 0.0–16.7 | 22 |
| AV surgery (%) | 10.3 | 0.0–38.9 | 22 |
| SAS resection (%) | 5.61 | 0.0–30.6 | 22 |
Pooled percentages are depicted for categorical variables and pooled mean/median with standard deviation in parentheses for continuous variables. The range of the mean/median values or percentages in the reporting studies is presented.
The number of concomitant anomalies and interventions are reported; therefore, the percentage can reach higher than 100% as multiple concomitant anomalies of cardiac interventions could be reported per patient.
AV: aortic valve; BAV: bicuspid aortic valve; NYHA: New York Heart Association; PA: pulmonary artery; SAS: subvalvular aortic stenosis; SD: standard deviation; SVAS: supravalvular aortic stenosis; VSD: ventricular septal defect.
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