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Severe Chronic Thromboembolic Pulmonary Hypertension (CTEPH) with Discordantly Near-Normal V/Q Scan

Severe Chronic Thromboembolic Pulmonary Hypertension (CTEPH) with Discordantly Near-Normal V/Q Scan▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

CT shows findings of severe pulmonary hypertension, including central pulmonary artery thrombi, webs, and stenoses, consistent with chronic pulmonary thromboembolism. Despite these findings, the perfusion and ventilation V/Q SPECT CT scan is near-normal, with only relatively small, non-wedge-shaped perfusion abnormalities. A patent foramen ovale (PFO) is also noted.

Key takeaways

In patients with chronic thromboembolic pulmonary hypertension (CTEPH), a near-normal V/Q scan does not exclude substantial central pulmonary artery pathology. This can occur if the peripheral vasculature is relatively spared, or if there is uniform elevation of pulmonary vascular resistance. V/Q scans reflect the relative distribution of pulmonary vascular resistances, which may not always correlate with the severity of central disease.

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