Table 2.
Characteristics of Patients with PAH: Cell Culture Experiments
| ID | Assay | Age (yr) | Sex | Race/Ethnicity | Diagnosis | BMPR2 Mutation | PAP (s/d/m) (mm Hg) | PVR (WU) | 6MWD (m) | PAH Medications |
|---|---|---|---|---|---|---|---|---|---|---|
| PAH-01 | Caspase WB | 27 | F | White non-Hispanic | IPAH | c.76 + 5G>GA probable mutation, not in dbSNP; may disrupt splicing | 110/49/69 | 12.11 | 420.6 | Sildenafil, tresprostinil, bosentan, iloprost |
| PAH-02 | Caspase WB | 33 | F | White non-Hispanic | FPAH | c.961C>T nonsense, exon 7 (kinase domain) | 87/29/48 | 9.74 | 288 | Bosentan, treprostinil, sildenafil, epoprostenol |
| PAH-03 | Caspase WB | 33 | F | Black/AA non-Hispanic | FPAH | c.1471C>T missense, exon 11 (kinase domain) | 75/33/48 | 15.57 | 326.1 | Epoprostenol, bosentan, sildenafil, treprostinil |
| PAH-04 | WB | 37 | M | White non-Hispanic | FPAH | c.1471C>T missense, exon 11 (kinase domain) | 119/51/77 | 14.22 | 309 | Sildenafil, sitaxsentan, ambrisentan, epoprostenol, imatinib (investigational drug) |
| PAH-05 | Caspase WB | 54 | F | White non-Hispanic | IPAH | NA | 100/45/60 | 11.96 | 296.3 | Sildenafil, epoprostenol, ambrisentan, bosentan |
| PAH-06 | Caspase WB | 27 | M | White non-Hispanic | IPAH | No | 90/51/68 | 11.38 | 423.7 | Bosentan, sildenafil, epoprostenol |
| PAH-07 | Caspase WB | 56 | M | White non-Hispanic | IPAH | No | 125/50/75 | 9.58 | 234.7 | Tadalafil, bosentan |
| PAH-08 | Caspase Matrigel WB | 32 | F | White non-Hispanic | IPAH | No | 68/38/49 | 15.34 | 238 | Bosentan, epoprostenol |
| PAH-09 | Matrigel WB | 40 | F | White non-Hispanic | IPAH | NA | 105/63/55 | 9.84 | 472.4 | Sildenafil, bosentan |
| PAH-10 | Matrigel WB | 56 | F | White non-Hispanic | FPAH | No | 110/55/75 | NA | 372.2 | Epoprostenol, bosentan, ambrisentan, sildenafil |
| PAH-11 | Matrigel | 55 | F | Black/AA non-Hispanic | IPAH | No | 89/41/53 | 12.29 | 273.4 | Sildenafil, bosentan, epoprostenol |
Definition of abbreviations: 6MWD = distance walked in 6 minutes; AA = African American; BMPR2 = bone morphogenetic protein receptor 2; dbSNP = Single Nucleotide Polymorphism database; FPAH = familial pulmonary arterial hypertension; ID = identification; IPAH = idiopathic pulmonary arterial hypertension; NA = data not available; PAH = pulmonary arterial hypertension; PAP (s/d/m) = pulmonary artery pressure (systolic/diastolic/mean); PVR = pulmonary vascular resistance (baseline Fick PVR); WB = Western immunoblot; WU = Wood units.
Hemodynamic data were obtained from catheterization study performed closest to transplantation.
PAH medications are listed according to total drug exposure during treatment period of follow-up, not necessarily in combination.