Clinical Presentations and Multimodal Imaging Diagnosis in Chronic Thromboembolic Pulmonary Hypertension

Figures

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  Figure 1. Pathophysiology of CTEPH. Abbreviations: CTEPH, chronic thromboembolic pulmonary hypertension; PA, pulmonary artery; RV, right ventricle.

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  Figure 2. CT findings in the case. CT imaging showed a dilated main PA and a dilated RV but no evidence of PE or lung parenchymal disease.

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  Figure 3. Echocardiographic findings associated with CTEPH. Note the dilated RV (A) and flattened interventricular septum (called the D-shaped LV, red arrows), which suggest RV pressure overloading (B). Pericardial effusion (asterisk) can be observed in advanced cases of CTEPH (B). Pulsed-wave Doppler image obtained from the RV outflow tract shows a midsystolic notch (arrow) and shortened acceleration time, which reflect increased pulmonary vascular resistance (C). Continuous-wave (CW) Doppler analysis at the tricuspid valve reveals a TR Vmax of 4.2 m/s, which strongly suggests significant PH (D).

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  Figure 4. CT findings suggestive of PH. (A) CT demonstrates a dilatation of the main pulmonary artery (MPA). The ratio of the MPA diameter to that of the ascending aorta (AA) is greater than 1. (B) CT scan obtained at a lower level shows RV dilatation. The ratio of the RV diameter to the LV diameter is greater than 1 at the midventricular level. Abbreviations: AA, ascending aorta; LV, left ventricle; MPA, main pulmonary artery; RA, right atrium; RV, right ventricle.

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  Figure 5. Distinct thrombus morphology for chronic (A) and acute thrombus (B). Note the eccentric thrombus (adhering to the vessel wall) in CTEPH (A); central filling defect in acute PE.

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  Figure 6. CT findings suggestive of CTEPH. Eccentric thrombus along the wall (A,B). Mosaic attenuation (C). Calcified thrombus (asterisks, (D,E)). Three-dimensional volume rendering image showing dilated main PA (arrowhead) and narrowing (white arrow) and pruning (yellow arrow) of PA (F).

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  Figure 7. PBV maps and corresponding 3D volume rendering images of pulmonary arteries in a CTEPH patient. PBV maps demonstrate perfusion defects (regions marked with an asterisk) in the right upper lobe (RUL) (A) and right lower lobe (RLL) (B). Three-dimensional volume rendering images demonstrate attenuation and occlusion of corresponding vessels.

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  Figure 8. V/Q scan findings demonstrating multiple perfusion defects in both lungs. Arrows indicate areas of showing perfusion defects. Abbreviations: ANT, anterior; R lat, right lateral; RPO, right posterior-oblique; POST, posterior; LPO, left posterior-oblique; L lat, left lateral.

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  Figure 9. The final diagnosis can be made via RHC (A) and DSA (B). Abbreviations: PCWP, pulmonary capillary wedge pressure.