CTICasesPulmonary Arterial Hypertension associated with Neurofibromatosis Type 1
[...]. Pulmonary Arterial Hypertension associated with Neurofibromatosis Type 1. CTICases. Published April 2, 2021. https://str-case-explorer.pages.dev/c/c5tqa
Imaging findings
CT in a patient with known NF-1 demonstrates multiple cutaneous neurofibromas on the skin, prominent pulmonary arteries indicating pulmonary hypertension, and a few scattered small pulmonary cysts. Perivascular haziness is noted.
Key takeaways
Neurofibromatosis type 1 (NF-1) is a rare but recognized cause of pulmonary arterial hypertension (PAH), occurring more frequently in NF-1 patients than in the general population. The mechanism is thought to involve a vasculopathy related to NF-1 gene dysfunction in vascular smooth muscle cells. NF-1-associated PAH should be considered in patients with NF-1 presenting with dyspnea or right heart strain.
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