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Updated: Oct 9, 2025

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Pulmonary arterial hypertension associated with pulmonary arteriovenous malformations and pulmonary veno-occlusive
Baukje M Zaaijer1, Nienke Duppen1, Brigitte W M Willemse2
1University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands.
Abstract:
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.
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