Diagnostic Considerations

Other WHO groups of pulmonary hypertension should be considered as coincident or alternative diagnoses. These include pulmonary arterial hypertension (PAH) (WHO group 1), pulmonary hypertension (WHO groups 2, 3, and 5), and other pulmonary arterial obstruction (eg, tumors of the pulmonary artery, pulmonary artery stenoses, arteritis, and mediastinal fibrosis).

Differential Diagnoses

Workup

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Media Gallery

Ventilation perfusion scan showing bilateral large wedge-shaped mismatched perfusion defects and areas of gray indicating decreased perfusion. Right-sided heart catheterization for this patient showed combined precapillary and postcapillary pulmonary hypertension and the following: right atrial pressure 10 mm Hg, right ventricular pressure 82/8 mm Hg, pulmonary artery wedge pressure 22 mm Hg, and pulmonary artery pressure 83/27 mm Hg with a mean of 49 mm Hg. He was referred to an expert center for pulmonary endarterectomy evaluation, where he underwent pulmonary angiography. Findings from the right side showed an occluded upper lobe anterior segment, a proximal web in the upper lobe, and disease in all lower segments. Findings from the left side showed an occluded superior segment of the lower lobe with disease in basal segments, proximal web in lingula, and intact upper lobe vessels. He underwent pulmonary endarterectomy with intraoperative University of California San Diego classification of thrombus as right 1 and left 2. Postoperatively, he has had dramatic improvement in his symptoms and is off all pulmonary arterial hypertension therapy.
Extensive pulmonary emboli are seen in both lungs, with a dominant, eccentric thrombus related to the left main pulmonary artery. More distal emboli were noted in the subsegmental pulmonary arteries (not shown). This is a 42-year-old woman with a history of multiple pulmonary emboli and protein S deficiency who initially presented with severe dyspnea (New York Heart Association functional class IV) and was initiated on multiple pulmonary arterial hypertension medications, including intravenous prostacyclin. She underwent pulmonary endarterectomy, and the images of the clots that were removed intraoperatively are shown in another image.
The heart is enlarged, with right atrial and ventricular enlargement in addition to reflux of contrast into the intrahepatic inferior vena cava. The main pulmonary artery itself measures approximately 3 cm. There was mosaic attenuation of both lungs, most pronounced related to the right-middle and upper lobes (not shown).
Mosaic pattern seen on expiratory images of a chest CT scan in a patient with chronic thromboembolic pulmonary hypertension. The same patient’s ventilation/perfusion scan is also shown. The areas of hypovascularity in blood vessels with clots cause areas of relative hypoperfusion, which appear darker than normal lung and give rise to the differential opacities seen on this image.
Intraoperative clot during pulmonary endarterectomy. This is a 42-year-old woman with a history of multiple pulmonary emboli and protein S deficiency who initially presented with severe dyspnea (New York Heart Association functional class IV) and was initiated on multiple pulmonary arterial hypertension medications, including intravenous prostacyclin. Preoperative right-sided heart catheterization showed right atrial pressure of 16 mm Hg, pulmonary artery pressure of 124/29 mm Hg with a mean of 67 mm Hg, cardiac output of 4.53, and pulmonary vascular resistance of 12 Wood units. Immediate postoperative right-sided heart catheterization showed right atrial pressure of 9 mm Hg, pulmonary artery pressure of 40/15 mm Hg with a mean of 25 mm Hg, cardiac output of 5.15, and pulmonary vascular resistance of 2.9 Wood units. She came off all her pulmonary arterial hypertension therapy immediately postoperatively and continues to do well.

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Group 4 Pulmonary Hypertension Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

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