CTICasesHereditary Hemorrhagic Telangiectasia-Associated Pulmonary Hypertension
Imaging findings
Serial chest radiographs and CT scans show progressive, severe dilatation of the main, right, and left pulmonary arteries over time. The pulmonary veins and left atrium are normal in caliber, and there are no large arteriovenous malformations (AVMs). High-resolution CT shows multiple small, centrilobular ground-glass smudges representing capillary proliferation and neo-vascularity. Right heart catheterization confirms severe pre-capillary pulmonary hypertension with a mean PAP of 59 mmHg and elevated pulmonary vascular resistance.
Key takeaways
Hereditary hemorrhagic telangiectasia (HHT or Osler-Weber-Rendu disease) is an autosomal dominant vascular disorder caused by mutations in ENG or ACVRL1. While HHT is famous for pulmonary AVMs, it is also associated with Group 1 pre-capillary pulmonary hypertension. This is histologically identical to idiopathic pulmonary arterial hypertension, driven by abnormal TGF-beta signaling, and can be precipitated or exacerbated by treating pulmonary AVMs.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Malignant MesotheliomaNeoplastic
- Pulmonary InfarctionVascular
- Intravascular Pulmonary MetastasesNeoplastic
- Rheumatoid Lung NoduleAutoimmune
- Congenital Pulmonary Airway MalformationCongenital
- Growing Teratoma SyndromeNeoplastic
- Pericardial LymphangiomaNeoplastic
- Pulmonary Langerhans Cell HistiocytosisILD
See all cases from January 11, 2018 →