Table 1.
Demographic and clinical characteristics of patients transitioned to oral treprostinil
| Characteristics | Value |
|---|---|
| No. patients (F/M) | 9 (7/2) |
| Age, median (range), years | 50 (34–72) |
| Actual body weight, median (range), kg | 74.8 (50.9–115.7) |
| Type of PAH, n | |
| IPAH | 5 |
| PAH-CTD | 2 |
| PAH-CHDa | 2 |
| WHO FC prior to transition, n | |
| 1 | 0 |
| 2 | 7 |
| 3 | 2 |
| 4 | 0 |
| 6MWD prior to transition, median (range), mb | 338 (53–515) |
| Reason for transition, n | |
| Intolerance of IV prostacyclin due to infection | 1 |
| Intolerance of SQ prostacyclin due to pain | 4 |
| Patient preference | 4 |
| Concomitant PAH therapy at time of transition | |
| None, n | 0 |
| PDE5i monotherapy, n | 5 |
| ERA monotherapy, n | 1 |
| PDE5i + ERA combination, n | 2 |
| No background therapy, n | 1 |
| Inhaled prostacyclin, n | 2 |
| Dose, mcg × QID | 72 |
| SQ prostacyclin, n | 5 |
| Dose, median (range), ng/kg/min | 40 (24–70) |
| IV prostacyclin, n | 2 |
| Dose, median (range), ng/kg/min | 44 (39–50) |
Note
ERA: endothelin receptor antagonists; F: females; IPAH: idiopathic PAH; IV: intravenous; M: males; PAH: pulmonary arterial hypertension; PAH-CHD: congenital heart disease–associated PAH; PAH-CTD: connective tissue disease–associated PAH; PDE5i: phosphodiesterase type-5 inhibitors; 6MWD: 6-minute walk distance; SQ: subcutaneous; WHO FC: World Health Organization functional class; QID = 4 times daily dosing.
a
For the 2 patients with PAH-CHD, 1 had a ventricular septal defect repair and the other had a late atrial septal defect closure.
b
6MWD not documented prior to transition in 1 patient.