C, control tissue; P, patient tissue; aPAH, associated PAH (collagen vascular disease); CTEPH, chronic thromboembolic pulmonary hypertension; PDE5i, phosphodiesterase type 5 inhibitor; ERA, endothelin receptor antagonist; Prostn, parenteral prostanoid; sGC, soluble guanylate cyclase; WHO, World Health Organization.
Patient | Diagnosis | Age | Sex | WHO class | PAH therapy | |||
Histology/immunoblots | C1 | Lobectomy (tumor) | 56 | M | II | None | ||
C2 | Unused transplant donor | 25 | M | I | None | |||
C3 | Unused transplant donor | 38 | F | I | None | |||
C4 | Unused transplant donor | 41 | F | I | None | |||
P1 | aPAH | 34 | F | III | PDE5i + ERA | |||
P2 | iPAH | 18 | M | IV | PDE5i + Prostn | |||
P3 | iPAH | 39 | F | IV | ERA + Prostn | |||
P4 | iPAH | 31 | F | III | ERA + Prostn | |||
P5 | iPAH | 42 | F | III | PDE5i + ERA + Prostn | |||
P6 | aPAH | 40 | M | IV | PDE5i + ERA + Prostn | SIRT3 variant | UCP2 variant | |
EVLP lung | 1 | aPAH | 62 | F | IV | PDE5i + ERA + Prostn | G/G | A/A |
2 | aPAH | 61 | F | IV | PDE5i + ERA + Prostn | G/A | A/A | |
3 | iPAH | 32 | F | IV | PDE5i + ERA + Prostn | G/G | A/A | |
4 | iPAH | 43 | F | IV | PDE5i + ERA + Prostn | G/A | G/G | |
5 | CTEPH | 60 | M | IV | sGC stim + Prostn | G/A | A/A |