Pulmonary arterial hypertension associated with pulmonary arteriovenous malformations and pulmonary veno-occlusive disease: A devastating combination

Baukje M Zaaijer, Nienke Duppen, Brigitte W M Willemse, Martijn V Verhagen, Marcus T R Roofthooft, Wim Timens, Rolf M F Berger, Johannes M Douwes*

*Bijbehorende auteur voor dit werk

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We describe a case of an adolescent male with the rare combination of pulmonary arterial hypertension (PAH) and pulmonary arteriovenous malformations (PAVM's) without confirmed hereditary hemorrhagic telangiectasia (HHT). The patient showed clinical deterioration on standard vasodilator therapy, leading us to question our initial diagnosis. Post-mortem evaluation confirmed the presence of pulmonary veno-occlusive disease of which no conclusive signs were recognized at diagnostic work-up. This case demonstrates the heterogeneity in the diseases causing PAH and shows that an unexpected treatment response should alert the physician to question the original diagnosis.

Originele taal-2English
Artikelnummer101564
Aantal pagina's6
TijdschriftRespiratory medicine case reports
Volume34
DOI's
StatusPublished - 2021

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