Advanced

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)
Please use this url to cite or link to this publication:
author
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
2014-10-01 07:24:56
date last changed
2017-11-05 04:03:19
@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},
  keyword      = {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},
  volume       = {48},
  year         = {2014},
}