Table 8.
Comparing clinical mimics CTEPH and PTTM.
| CTEPH | PTTM | |
|---|---|---|
| Clinical presentation | ||
| Clinical progression | Chronic | Acute to subacute |
| Dyspnea* | Common | Common |
| Cough* | Less common | Common |
| Radiographic features | ||
| Chest CT† | Mosaicism Wedge-shaped infarcts Prominence of bronchial artery circulation | Ground-glass opacities Nodules Mediastinal/hilar adenopathy Septal thickening |
| Hemodynamics ‡ | ||
| mPAP, mmHg (median) | 47 (n = 669) | 48 (n = 20) |
| PCWP, mmHg (median) | – | 12 (n = 14) |
| PVR, dynes*s*cm−5 (median) | 709 (n = 604) | 928 (n = 9) |
| Cardiac index, L/min/m2 (median) | 2.2 (n = 632) | 2 (n = 8) |
| Histopathology § | ||
| Thromboemboli | Y | Y |
| Fibrocellular intimal proliferation | Y | Y |
| Involvement of pulmonary arterioles | Y | Y |
| Involvement of pulmonary venules | Y | Y |
| Presence of tumor cells | N | Y |
*
Symptoms of dyspnea can be seen in both; while cough is atypical in CTEPH, it is commonly noted in PTTM. Absence of these symptoms does not preclude either of the two disease entities.
†
Radiographic information regarding CTEPH obtained from Gopalan et al.40 Radiographic features of PTTM obtained from review of all cases included in this systematic review with radiographic information available.
‡
Hemodynamic data obtained from Pepke-Zaba et al. CTEPH prospective international registry.43
§
Histopathology information regarding CTEPH obtained from Lang et al.42