Age-related changes among 25 patients with congenital cardiac left-to-right shunts and irreversible plexogenic pulmonary arteriopathy

Among patients with congenital heart disease and plexogenic pulmonary arteriopathy, histopathological differences were observed between those surviving into adulthood and those dying in childhood/adolescence. Plexiform lesions in adults undergo remodeling from a cellular morphology to an involuted morphology. It is unclear whether this simply represents the natural progression of plexiform lesions or also confers a survival advantage.