CTICasesGranulomatous (Sarcoid-Associated) Pulmonary Veno-Occlusive Disease
Imaging findings
A man with remote neurosarcoidosis, off immunosuppression for years, presented with progressive dyspnea and scattered fuzzy nodules that over months evolved into worsening interlobular septal thickening, peribronchial cuffing, small effusions, and mediastinal nodes, with a pattern resembling edema despite a normal left heart. A VQ scan showed multiple bilateral mismatched defects mimicking chronic thromboembolic disease, and right heart catheterization pressures rose from mildly elevated to a mean over 70 before lung transplant. Explant histology showed inflammation and occlusion of pulmonary veins by collagen with granulomas, with normal arteries.
Key takeaways
Pulmonary veno-occlusive disease can present as apparent interstitial edema (septal thickening, effusions, nodes) with a normal left heart and rapidly progressive pulmonary hypertension, and importantly it can produce mismatched perfusion defects on VQ scanning that mimic chronic thromboembolic disease. Granulomatous pulmonary venous occlusion is a rare but recognized association with sarcoidosis, and by the time pulmonary hypertension develops the disease is typically irreversible. Combined CT angiography and VQ imaging are complementary in evaluating pulmonary hypertension.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Uncorrected Intracardiac Total Anomalous Pulmonary Venous Return with Eisenmenger PhysiologyCongenital
- Ponytail Artifact Mimicking a Hilar NoduleArtifact
- Aneurysm of the Membranous Interventricular SeptumCongenital
- Cardiac LipomaNeoplastic
- Posterior Mediastinal LipomaNeoplastic
- High-Grade Aortic Coarctation (Near-Atresia)Congenital
- Recurrent Sternal DehiscenceIatrogenic
- Tracheal ChondrosarcomaNeoplastic
See all cases from January 7, 2021 →
Related Vascular cases
- COVID-19 Thrombotic State with Myocardial Infarction and LV Thrombus
- In-Situ Pulmonary Artery Thrombus after Radiation
- Fleischner Sign of Acute Pulmonary Embolism
- Enlarging Pseudoaneurysm of a Calcified Aortic False Lumen
- Type 2 Endoleak after TEVAR
- Pulmonary Veno-Occlusive Disease / Pulmonary Capillary Hemangiomatosis