Table 1.
General characteristics of the included studies
| Study ID | PH types for data extraction | Area | Participants (N) | mutation /non-mutation (N/N) |
Male/Female (N/N) |
Outcomes | Period |
|---|---|---|---|---|---|---|---|
| Austin 2009 [8] | HPAH, IPAH | US | 147 | 106/41 | 43/104 | death or lung transplantation | up to date as of 2009.03 |
| Bruggen 2016 [12] | HPAH, IPAH | Netherlands | 95 | 28/67 | 23/72 | death or heart/lung transplantaton | 1995.03 to 2014.10 |
| Chida 2012 [20] | IPAH, HPAH | Japan and China | 54 | 18/36 | 24/30 | death | 1995.01.01 to 2011.03.31 |
| Elliott 2006 [21] | HPAH, IPAH | US | 67 | 27/40 | 14/53 | NA | 1994.07 - |
| Gamou 2017 [14] | HPAH | Japan | 117 | 39/78 | 31/86 | NA | 2015 |
| Ghigna 2016 [10] | HPAH, IPAH | French | 44 | 23/21 | 16/28 | lung transplantation | 2005–2014 |
| Girerd 2010 [9] | HPAH, IPAH | French | 382 | 115/267 | 113/269 | death or lung transplantation | 2004.01.01–2010.04.01 |
| Isobe 2016 [18] | HPAH, IPAH | Japan | 59 | 23/36 | 17/42 | death | 2000.08–2015.10 |
| Kabata 2013 [13] | HPAH, IPAH | Japan | 49 | 18/31 | 17/32 | death or lung transplantation | 1999.10–2007.03 |
| Liu 2012 [11] | HPAH, IPAH | China | 305 | 50/255 | 87/218 | death | 2006.01.01–2010.08.31 |
| Mutlu 2016 [23] | IPAH, CHD-PAH | Turkish | 43 | 1/42 | 21/22 | NA | 2011–2012 |
| Navas 2016 [19] | HPAH, IPAH | Spain | 165 | 24/141 | 41/124 | death or lung transplantation | 2011.01–2015.05.01 |
| Pfarr 2011 [17] | HPAH, IPAH | Germany | 228 | 49/179 | 62/166 | NA | 2006.01–2009.12 |
| Pousada 2014 [22] | IPAH and Associated PAH | Spain | 41 | 9/32 | 21/20 | NA | 14 months |
| Rosenzweig 2008 [24] | HPAH, IPAH | US | 147 | 23/124 | 49/98 | NA | 1991–2005 |
| Sztrymf 2008 [15] | HPAH, IPAH | French | 233 | 68/165 | 66/167 | death or lung transplantation | 2004.01–2007.06 |
| Yang 2018 [16] | HPAH, IPAH | China | 185 | 56/129 | 48/137 | NA | 2016–2017 |
NA not available, HAPH heritable PAH, IPAH idiopathic PAH