CTICasesHepatopulmonary Syndrome Type 2 with Pulmonary Vascular Nodules
Imaging findings
CT in a patient with hypoplastic left heart syndrome status post Fontan repair, chronic liver disease, and cirrhosis demonstrates multiple subpleural pulmonary nodules over five years, some containing internal dilated tortuous vascular channels, with arterial enhancement and delayed washout. Findings are consistent with type 2 hepatopulmonary syndrome with macroscopic nodular vascular malformations.
Key takeaways
Hepatopulmonary syndrome (HPS) is a triad of liver disease, intrapulmonary vascular dilatation, and hypoxemia. Type 2 HPS is characterized by discrete vascular malformations (nodular lesions) rather than diffuse microscopic dilatations. In patients with Fontan-associated liver disease, these nodules can be mistaken for metastases, but their long-term stability, internal vascularity, and arterial-phase enhancement pattern are diagnostic clues.
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