CTICasesIdiopathic Pulmonary Veno-Occlusive Disease
Imaging findings
CT chest in a 15-year-old boy shows severe right heart and pulmonary artery dilation, right ventricular wall thickening, small bilateral pleural effusions, and basal septal thickening, with severe pulmonary hypertension and normal wedge pressures confirmed on catheterization. The patient deteriorated rapidly after vasodilator therapy, requiring ECMO.
Key takeaways
Idiopathic pulmonary veno-occlusive disease (PVOD) is a rapidly progressive and devastating vasculopathy in children. The classic presentation includes septal lines, effusions, and severe pulmonary hypertension with normal wedge pressures. Administration of pulmonary vasodilators is contraindicated as it can precipitate life-threatening pulmonary edema.
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