Patient A |
46-year-old female with IPAH |
WHO-FC III, 6MWD 320 m, mRAP 8 mmHg, mPAP 56 mmHg and cardiac index 2.6 L·min−1·m−2 |
Rapidly progressive illness |
Patient goals: survival at any cost |
This patient received rapid sequential subcutaneous treprostinil and PDE5 inhibitor |
Patient B |
38-year-old female with scleroderma PAH |
WHO-FC III, 6MWD 350 m, mRAP 12 mmHg, mPAP 54 mmHg and cardiac index 2.2 L·min−1·m−2 |
Severe right ventricular dilation with severe systolic impairment |
Patient goals: to increase exercise capacity and improve symptoms |
This patient did not wish to undergo i.v. therapy; she began oral treatment with combined PDE5 inhibitor and ERA. She subsequently deteriorated and was started on i.v. epoprostenol |
IPAH: idiopathic pulmonary arterial hypertension (PAH); WHO: World Health Organization; FC: functional class; 6MWD: 6-min walking distance; mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; PDE5: phosphodiesterase type-5; ERA: endothelin receptor antagonist.