In adults with congenital heart septal defects, PAH is common and associated with worse symptoms and clinical deterioration, even after defect closure.
Supports PAH screening in adults with septal defects regardless of closure; leaves open effects of targeted therapy on progression.
In this selected population of adults with congenital heart disease, PAH was common and predisposed to more symptoms and further clinical deterioration, even among patients with previous defect closure and patients who had not developed Eisenmenger's physiology.
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Engelfriet et al. (2006) studied this question.
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