Abstract
It has long been debated whether patients with atrial septal defect (ASD) and Eisenmenger syndrome have idiopathic pulmonary
arterial hypertension (IPAH) with incidental ASD or severe pulmonary hypertension on the basis of their ASD shunt magnitude alone.
Severe pulmonary hypertension and plexogenic arteriopathy developed for 6% patients with ASD. Pulmonary vascular structure was
analyzed in the lungs of child 1 year 1 month age old using lung biopsy tissue and autopsy material Patients with ASD and
Eisenmenger syndrome have a clinical and morphological presentation similar to that of IPAH patients. Our patient with ASD have
histopathological presentation similar to that of IPAH. Althouh plexogenic arteriopathy of small pulmonary arteries was found in the
present study we had not obstructive features characterizing plexogenic arteriophatic. It was hypothesized that this plexogenic arteriopathic
for child have another character - congenital form. He underwent surgery (Potts) and died after 5 days. This patient may
carry a genetic mutation predisposing to the development of pulmonary hypertension and advanced obstructive lesions in plexiform
structures which may ОП1У become clinically apparent when triggered by аn external stimulus.
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