Table 1.
General patient characteristics.
| SScPAH | IPAH | Control | |
|---|---|---|---|
| N = 5 | N = 9 | N = 4 | |
| Age, yrs | 47 ± 4 | 47 ± 4 | 31 ± 4 |
| Male/Female no. | 1/4 | 2/7 | — |
| Survival | 1.1 ± 0.5 | 3.7 ± 0.9 | — |
| Heart rate | 85 ± 3 | 78 ± 5 | |
| mPpa, mmHg | 46 ± 7 | 62 ± 4 | |
| PCWP, mmHg | 6 ± 2 | 5 ± 2 | |
| PVR, dynes·s·cm−5 | 1221 ± 691 | 1157 ± 144 | |
| CI, l/min·m2 | 2.3 ± 0.9 | 2.3 ± 0.5 | |
| Systolic blood pressure | 105 ± 2 | 118 ± 8 | |
| Diastolic blood pressure | 69 ± 6 | 73 ± 5 | |
| TLC, % | 89 ± 5 | 92 ± 5 | |
| DLCO, % | 42 ± 6 | 64 ± 6 | |
| Therapy at time of death | |||
| Prostacycline (n) | 4 | 7 | |
| ERA (n) | 1 | 2 | |
| PDE-5 inhibitor (n) | 1 | 0 | |
| ABS | 1 |
Values expressed as mean ± SE or otherwise as stated. Abbreviations: ABS: atrial balloon septostomy; CI: cardiac index; DLCO%: percentage of predicted of the diffusion capacity of the lung for carbon monoxide; ERA: endothelin receptor antagonist; mPpa: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PDE-5: phosphodiesterase 5; PVR: pulmonary vascular resistance; IPAH: idiopathic pulmonary arterial hypertension; SScPAH: systemic sclerosis-associated pulmonary arterial hypertension; TLC%: percentage of predicted total long capacity.