Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry
Background Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010. We sought to determine mortality in PAH in the modern era using the PHAR (Pulmonary Hypertension Association Registry). Methods and Results W...
| Main Authors: | , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2022-05-01
|
| Series: | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| Subjects: | |
| Online Access: | https://www.ahajournals.org/doi/10.1161/JAHA.121.024969 |
| _version_ | 1811267004444704768 |
|---|---|
| author | Kevin Y. Chang Sue Duval David B. Badesch Todd M. Bull Murali M. Chakinala Teresa De Marco Robert P. Frantz Anna Hemnes Stephen C. Mathai Erika Berman Rosenzweig John J. Ryan Thenappan Thenappan |
| author_facet | Kevin Y. Chang Sue Duval David B. Badesch Todd M. Bull Murali M. Chakinala Teresa De Marco Robert P. Frantz Anna Hemnes Stephen C. Mathai Erika Berman Rosenzweig John J. Ryan Thenappan Thenappan |
| author_sort | Kevin Y. Chang |
| collection | DOAJ |
| description | Background Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010. We sought to determine mortality in PAH in the modern era using the PHAR (Pulmonary Hypertension Association Registry). Methods and Results We identified all adult patients with PAH enrolled in the PHAR between September 2015 and September 2020 (N=935). We used Kaplan‐Meier survival analysis and Cox proportional hazards models to assess mortality at 1, 2, and 3 years. Patients were stratified based on disease severity by 3 validated risk scores. In treatment‐naïve patients, we compared survival based on initial treatment strategy. The median age was 56 years (44–68 years), and 76% were women. Of the 935 patients, 483 (52%) were ≤6 months from PAH diagnosis. There were 121 deaths (12.9%) during a median follow‐up time of 489 days (281–812 days). The 1‐, 2‐, and 3‐year mortality was 8% (95% CI, 6%–10%), 16% (95% CI, 13%–19%), and 21% (95% CI, 17%–25%), respectively. When stratified into low‐, intermediate‐, and high‐risk PAH, the mortality at 1, 2, and 3 years was 1%, 4% to 6%, and 7% to 11% for low risk; 7% to 8%, 11% to 16%, and 18% to 20% for intermediate risk; and 12% to 19%, 22% to 38%, and 28% to 55% for high risk, respectively. In treatment‐naïve patients, initial combination therapy was associated with better 1‐year survival (adjusted hazard ratio, 0.43 [95% CI, 0.19–0.95]; P=0.037). Conclusions Mortality in the intermediate‐ and high‐risk patients with PAH remains unacceptably high in the PHAR, suggesting the importance for early diagnosis, aggressive use of available therapies, and the need for better therapeutics. |
| first_indexed | 2024-04-12T20:54:40Z |
| format | Article |
| id | doaj.art-6095b01f3f084472a0180b96e7214fe7 |
| institution | Directory Open Access Journal |
| issn | 2047-9980 |
| language | English |
| last_indexed | 2024-04-12T20:54:40Z |
| publishDate | 2022-05-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| spelling | doaj.art-6095b01f3f084472a0180b96e7214fe72022-12-22T03:17:02ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802022-05-0111910.1161/JAHA.121.024969Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association RegistryKevin Y. Chang0Sue Duval1David B. Badesch2Todd M. Bull3Murali M. Chakinala4Teresa De Marco5Robert P. Frantz6Anna Hemnes7Stephen C. Mathai8Erika Berman Rosenzweig9John J. Ryan10Thenappan Thenappan11Department of Medicine University of Minnesota Minneapolis MNDepartment of Medicine University of Minnesota Minneapolis MNDepartment of Medicine University of Colorado Denver CODepartment of Medicine University of Colorado Denver CODepartment of Medicine Washington University St. Louis MODepartment of Medicine University of California San Francisco San Francisco CADepartment of Medicine Mayo Clinic Rochester MNDepartment of Medicine Vanderbilt University Nashville TNDepartment of Medicine Johns Hopkins University Baltimore MDDepartment of Medicine Columbia University New York NYDepartment of Medicine University of Utah Salt Lake City UTDepartment of Medicine University of Minnesota Minneapolis MNBackground Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010. We sought to determine mortality in PAH in the modern era using the PHAR (Pulmonary Hypertension Association Registry). Methods and Results We identified all adult patients with PAH enrolled in the PHAR between September 2015 and September 2020 (N=935). We used Kaplan‐Meier survival analysis and Cox proportional hazards models to assess mortality at 1, 2, and 3 years. Patients were stratified based on disease severity by 3 validated risk scores. In treatment‐naïve patients, we compared survival based on initial treatment strategy. The median age was 56 years (44–68 years), and 76% were women. Of the 935 patients, 483 (52%) were ≤6 months from PAH diagnosis. There were 121 deaths (12.9%) during a median follow‐up time of 489 days (281–812 days). The 1‐, 2‐, and 3‐year mortality was 8% (95% CI, 6%–10%), 16% (95% CI, 13%–19%), and 21% (95% CI, 17%–25%), respectively. When stratified into low‐, intermediate‐, and high‐risk PAH, the mortality at 1, 2, and 3 years was 1%, 4% to 6%, and 7% to 11% for low risk; 7% to 8%, 11% to 16%, and 18% to 20% for intermediate risk; and 12% to 19%, 22% to 38%, and 28% to 55% for high risk, respectively. In treatment‐naïve patients, initial combination therapy was associated with better 1‐year survival (adjusted hazard ratio, 0.43 [95% CI, 0.19–0.95]; P=0.037). Conclusions Mortality in the intermediate‐ and high‐risk patients with PAH remains unacceptably high in the PHAR, suggesting the importance for early diagnosis, aggressive use of available therapies, and the need for better therapeutics.https://www.ahajournals.org/doi/10.1161/JAHA.121.024969pulmonary hypertensionright ventriclesurvivalUnited Statesvasodilators |
| spellingShingle | Kevin Y. Chang Sue Duval David B. Badesch Todd M. Bull Murali M. Chakinala Teresa De Marco Robert P. Frantz Anna Hemnes Stephen C. Mathai Erika Berman Rosenzweig John J. Ryan Thenappan Thenappan Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease pulmonary hypertension right ventricle survival United States vasodilators |
| title | Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry |
| title_full | Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry |
| title_fullStr | Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry |
| title_full_unstemmed | Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry |
| title_short | Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry |
| title_sort | mortality in pulmonary arterial hypertension in the modern era early insights from the pulmonary hypertension association registry |
| topic | pulmonary hypertension right ventricle survival United States vasodilators |
| url | https://www.ahajournals.org/doi/10.1161/JAHA.121.024969 |
| work_keys_str_mv | AT kevinychang mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT sueduval mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT davidbbadesch mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT toddmbull mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT muralimchakinala mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT teresademarco mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT robertpfrantz mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT annahemnes mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT stephencmathai mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT erikabermanrosenzweig mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT johnjryan mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry AT thenappanthenappan mortalityinpulmonaryarterialhypertensioninthemoderneraearlyinsightsfromthepulmonaryhypertensionassociationregistry |