Monitoring

Patients with mild PS do not require therapy but need to be monitored in an outpatient setting for progression of disease. While mild PS is thought of as a static lesion, studies using colour echocardiography suggest that infancy is the highest risk period for progression.[12] Therefore, patients need to be monitored annually until 4 years of age, after which clinical visits can range from every 3 to 5 years into adulthood. Less frequent follow-up may be appropriate for adult patients.[14]

Given the potential neurodevelopmental impacts associated with PS, incorporating comprehensive genetic evaluation and neurodevelopmental surveillance into the routine monitoring protocol is crucial, especially for patients who have undergone cardiac surgery or present with risk factors for developmental delays. This ensures a comprehensive approach to care that addresses all aspects of the patient's well-being.[7]

Clinical visits should consist of history, physical examination, ECG, echocardiography, and an exercise test when indicated.[13]

For those who have undergone an interventional procedure, follow-up will depend on the severity of stenosis after dilation. In general, there will be visits at 6 to 12 months, 5 years, and then every 10 years post-intervention.

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