Table 1.
Characterization of 11 Major PAH Registries
| Registry | Study Cohort | Number | Time Period | Study Design | Study cohort | IPAH/HPAH vs. APAH |
|---|---|---|---|---|---|---|
| NIH | IPAH | 187 | 1981-1985 | Prospective | Incident cases | NA |
| Chinese | IPAH/HPAH | 72 | 1999-2004 | Prospective | Incident cases | NA |
| COMPERA§ | IPAH/HPAH | 587 | 2007-2011 | Prospective | Incident cases | NA |
| French | Group I PAH | 674 | 2002-2003 | Prospective | Incident and prevalent cases | 39% vs. 61% |
| Mayo | Group I PAH | 484 | 1995-2004 | Prospective | Incident and prevalent cases | |
| New Chinese | Group I PAH | 956 | 2008-2011 | Prospective | Incident cases | 35% vs. 65% |
| PHC | Group I PAH | 578 | 1982-2006 | Retrospective & Prospective |
Incident and prevalent cases | 48% vs. 52% |
| REVEAL | Group I PAH | 3515 | 2006-2009 | Prospective | Incident and prevalent cases | 46% vs. 54% |
| Scottish-SMR | Group I PAH | 374 | 1986-2001 | Retrospective | Incident cases | 47% vs. 54% |
| Spanish | Group I PAH CTEPH |
866/162 | 1998-2008 | Retrospective & Prospective |
Incident and prevalent cases | 36% vs. 64% |
| UK and Ireland | IPAH, HPAH, and Anorexigen-APAH |
482 | 2001-2009 | Incident cases | 98.3% vs. 1.7% |
§
- COMPERA registry enrolled patients with any WHO group PH. The data presented here is for the incident IPAH cohort only.
NIH – national institutes of health, PHC – pulmonary hypertension connections, PAH – pulmonary arterial hypertension, IPAH – idiopathic PAH, and CTEPH – chronic thromboembolic pulmonary hypertension, HPAH – heritable PAH, and APAH – associated PAH.