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Prognosis and response to first-line single and combination therapy in pulmonary arterial hypertension

Kylhammar, David LU ; Persson, Liselotte ; Hesselstrand, Roger LU and Rådegran, Göran LU (2014) In Scandinavian Cardiovascular Journal 48(4). p.223-233
Abstract
Objectives. To investigate survival, treatment escalation, effects of first-line single- and first-line combination therapy and prognostic markers in idiopathic- (IPAH), hereditary- (HPAH) and connective tissue disease-associated (CTD-PAH) pulmonary arterial hypertension (PAH). Design. Retrospective analysis of medical journals from PAH patients at Skane University Hospital 2000-2011. Results. 1-, 2- and 3-year survival was 87%, 67%, and 54%, respectively, for the entire population, but worse (p = 0.003) in CTD-PAH than IPAH/HPAH. After 1, 2 and 3 years, 58%, 41% and 24% of patients starting on single therapy were alive on single therapy. 37.5% of patients on first-line single therapy received escalated treatment at first follow-up.... (More)
Objectives. To investigate survival, treatment escalation, effects of first-line single- and first-line combination therapy and prognostic markers in idiopathic- (IPAH), hereditary- (HPAH) and connective tissue disease-associated (CTD-PAH) pulmonary arterial hypertension (PAH). Design. Retrospective analysis of medical journals from PAH patients at Skane University Hospital 2000-2011. Results. 1-, 2- and 3-year survival was 87%, 67%, and 54%, respectively, for the entire population, but worse (p = 0.003) in CTD-PAH than IPAH/HPAH. After 1, 2 and 3 years, 58%, 41% and 24% of patients starting on single therapy were alive on single therapy. 37.5% of patients on first-line single therapy received escalated treatment at first follow-up. First-line combination therapy more greatly decreased pulmonary vascular resistance index (PVRI, p = 0.017) than first-line single therapy. Only first-line combination therapy improved (p = 0.042) cardiac index (CI). Higher mean right atrial pressure (MRAP, p = 0.018), MRAP/CI (p = 0.021) and WHO functional class (p < 0.001) and lower 6-min walking distance (6MWD, p = 0.001) at baseline, and higher PVRI (p = 0.008) and lower 6MWD (p = 0.004) at follow-up were associated with worse outcome. Conclusions. We confirm improved survival with PAH-targeted therapies. Survival is still poor and early treatment escalation frequently needed. First-line combination therapy may more potently improve haemodynamics. MRAP/CI may represent a new prognostic marker in PAH. (Less)
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author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
combination therapy, human, prognosis, pulmonary arterial hypertension, survival
in
Scandinavian Cardiovascular Journal
volume
48
issue
4
pages
223 - 233
publisher
Taylor & Francis
external identifiers
  • wos:000340233800005
  • scopus:84904619344
ISSN
1651-2006
DOI
10.3109/14017431.2014.931595
language
English
LU publication?
yes
id
3d684a98-25d1-4305-8fb2-914c6e2c490a (old id 4659315)
date added to LUP
2016-04-01 13:53:06
date last changed
2022-01-27 21:38:15
@article{3d684a98-25d1-4305-8fb2-914c6e2c490a,
  abstract     = {{Objectives. To investigate survival, treatment escalation, effects of first-line single- and first-line combination therapy and prognostic markers in idiopathic- (IPAH), hereditary- (HPAH) and connective tissue disease-associated (CTD-PAH) pulmonary arterial hypertension (PAH). Design. Retrospective analysis of medical journals from PAH patients at Skane University Hospital 2000-2011. Results. 1-, 2- and 3-year survival was 87%, 67%, and 54%, respectively, for the entire population, but worse (p = 0.003) in CTD-PAH than IPAH/HPAH. After 1, 2 and 3 years, 58%, 41% and 24% of patients starting on single therapy were alive on single therapy. 37.5% of patients on first-line single therapy received escalated treatment at first follow-up. First-line combination therapy more greatly decreased pulmonary vascular resistance index (PVRI, p = 0.017) than first-line single therapy. Only first-line combination therapy improved (p = 0.042) cardiac index (CI). Higher mean right atrial pressure (MRAP, p = 0.018), MRAP/CI (p = 0.021) and WHO functional class (p &lt; 0.001) and lower 6-min walking distance (6MWD, p = 0.001) at baseline, and higher PVRI (p = 0.008) and lower 6MWD (p = 0.004) at follow-up were associated with worse outcome. Conclusions. We confirm improved survival with PAH-targeted therapies. Survival is still poor and early treatment escalation frequently needed. First-line combination therapy may more potently improve haemodynamics. MRAP/CI may represent a new prognostic marker in PAH.}},
  author       = {{Kylhammar, David and Persson, Liselotte and Hesselstrand, Roger and Rådegran, Göran}},
  issn         = {{1651-2006}},
  keywords     = {{combination therapy; human; prognosis; pulmonary arterial hypertension; survival}},
  language     = {{eng}},
  number       = {{4}},
  pages        = {{223--233}},
  publisher    = {{Taylor & Francis}},
  series       = {{Scandinavian Cardiovascular Journal}},
  title        = {{Prognosis and response to first-line single and combination therapy in pulmonary arterial hypertension}},
  url          = {{http://dx.doi.org/10.3109/14017431.2014.931595}},
  doi          = {{10.3109/14017431.2014.931595}},
  volume       = {{48}},
  year         = {{2014}},
}