Objective: MR imaging has proven accurate in identifying patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, we know of no attempt to distinguish patients with CTEPH from patients with other causes of pulmonary arterial hypertension (PAH). Primary pulmonary hypertension (PPH) is the disease most frequently confused with CTEPH. We examined patients with CTEPH or PPH and control subjects to identify MR imaging features that might distinguish CTEPH from PPH, to compare the accuracy of MR angiography (MRA) with that of radionuclide scanning, and to determine the cardiac and pulmonary vascular measurements in these groups.
Subjects and methods: T1-weighted and two-dimensional multiplanar spoiled gradient-recalled scans were obtained in 30 patients with CTEPH who had undergone conventional pulmonary angiography, 10 patients with PPH, and 13 control subjects with no known vascular disease. Ventilation-perfusion scans were available in all patients with CTEPH and in six of the 10 patients with PPH. The MR scans were assessed independently by two radiologists who evaluated the appearance of segmental vessels and noted the presence of mosaic perfusion. Cardiac chambers and pulmonary vessels were measured on T1-weighted spin-echo scans.
Results: The two radiologists used MR angiograms to reliably distinguish between patients with CTEPH and those with PPH. The accuracy of MR angiograms matched that of ventilation-perfusion scans (92%). On T1-weighted scans, the two radiologists used cardiac and pulmonary vascular measurements to distinguish patients with PAH from control subjects but failed to distinguish between patients with CTEPH and those with PPH.
Conclusion: MRA is useful in distinguishing patients with CTEPH from those with PPH. In this population, MRA had an accuracy that was identical to that of radionuclide scanning. Vascular and cardiac measurements made on MR scans reliably identified patients with PAH but did not distinguish between patients with CTEPH and those with PPH.