Prognosis

Mild pulmonary stenosis (PS)

Survival in untreated patients is equivalent to that of the general population.[22]

While mild PS is thought of as a static lesion, modern studies using color echocardiography suggest that infancy is the highest-risk period for progression.[12]

Moderate/severe/critical PS

Critical PS is fatal in the neonate without intervention.[9]

Percutaneous valvuloplasty produces excellent short- and long-term results in reducing the pulmonary valve obstruction.[23] Data from more than 26 institutions within the Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry resulted in an overall acute decrease in peak systolic gradients from 71 to 28 mmHg.[24]

Long-term results of 860 patients followed up for 14 years after balloon valvuloplasty showed that 4 out of 5 were free from clinically significant PS.[23] For neonates with critical PS, the rate of technical success for this procedure is about 90%, with a 5% complication rate. The freedom from reintervention rate (surgical or catheterization) has been estimated to be 79%. The study identified the following independent risk factors for suboptimal outcome: higher initial pulmonary valvular gradient, high early residual gradient, dysplastic valve, and younger age at intervention.

In a study on patients with nondysplastic valves, surgical valvuloplasty decreased the mean pressure gradient significantly more compared with balloon valvuloplasty.[25] In addition, surgical valvuloplasty was associated with fewer reinterventions but a longer hospital stay and led more frequently to moderate pulmonary valve insufficiency following the intervention.

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