PMID- 17079256
OWN - NLM
STAT- MEDLINE
DCOM- 20070516
LR  - 20181201
IS  - 0903-1936 (Print)
IS  - 0903-1936 (Linking)
VI  - 29
IP  - 3
DP  - 2007 Mar
TI  - Addition of sildenafil to bosentan monotherapy in pulmonary arterial 
      hypertension.
PG  - 469-75
AB  - Combination therapy has been recommended for the treatment of pulmonary arterial 
      hypertension (PAH). However, there is scant information on combination therapy 
      after failure of monotherapy, particularly in patients with 
      scleroderma-associated PAH (PAH-SSD). From a group of 82 consecutive patients 
      with PAH who received initial bosentan monotherapy, a total of 13 idiopathic PAH 
      (IPAH) and 12 PAH-SSD patients requiring additional therapy with sildenafil were 
      studied. Sildenafil was added for clinical deterioration based upon symptoms, New 
      York Heart Association (NYHA) classification or 6-min walk distance (6MWD). 
      Clinical data and haemodynamics were collected at baseline. Assessments were made 
      at 1-3-month intervals. At baseline, there were no differences in demographics, 
      NYHA classification, haemodynamics or 6MWD between the two groups. After 
      initiation of bosentan, both groups experienced clinical improvement but 
      ultimately deteriorated (median time to monotherapy failure 792 versus 458 days 
      for IPAH and PAH-SSD patients, respectively). After addition of sildenafil, more 
      IPAH patients tended to improve in NYHA class (five out of 13 versus two out of 
      12) and walked further (mean difference in 6MWD 47+/-77 m versus -7+/-40 m) 
      compared with PAH-SSD patients. In conclusion, addition of sildenafil after 
      bosentan monotherapy failure improved New York Heart Association class and 6-min 
      walk distance in idiopathic pulmonary arterial hypertension patients but failed 
      to improve either parameter in scleroderma-associated pulmonary arterial 
      hypertension patients. Additional studies are needed to assess the tolerability 
      and efficacy of this combination in patients with scleroderma-associated 
      pulmonary arterial hypertension.
FAU - Mathai, S C
AU  - Mathai SC
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 1830 
      East Monument Street, 5th Floor, Baltimore, MD 21205, USA.
FAU - Girgis, R E
AU  - Girgis RE
FAU - Fisher, M R
AU  - Fisher MR
FAU - Champion, H C
AU  - Champion HC
FAU - Housten-Harris, T
AU  - Housten-Harris T
FAU - Zaiman, A
AU  - Zaiman A
FAU - Hassoun, P M
AU  - Hassoun PM
LA  - eng
PT  - Journal Article
DEP - 20061101
PL  - England
TA  - Eur Respir J
JT  - The European respiratory journal
JID - 8803460
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Piperazines)
RN  - 0 (Purines)
RN  - 0 (Sulfonamides)
RN  - 0 (Sulfones)
RN  - 0 (Vasodilator Agents)
RN  - BW9B0ZE037 (Sildenafil Citrate)
RN  - Q326023R30 (Bosentan)
SB  - IM
CIN - Eur Respir J. 2007 Mar;29(3):432-4. PMID: 17329488
MH  - Adult
MH  - Aged
MH  - Antihypertensive Agents/adverse effects/*therapeutic use
MH  - Bosentan
MH  - Dose-Response Relationship, Drug
MH  - Drug Therapy, Combination
MH  - Exercise Test/drug effects
MH  - Female
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy
MH  - Lung Volume Measurements
MH  - Male
MH  - Middle Aged
MH  - Piperazines/adverse effects/*therapeutic use
MH  - Pulmonary Wedge Pressure/drug effects
MH  - Purines/adverse effects/therapeutic use
MH  - Scleroderma, Systemic/complications/drug therapy
MH  - Sildenafil Citrate
MH  - Sulfonamides/adverse effects/*therapeutic use
MH  - Sulfones/adverse effects/*therapeutic use
MH  - Treatment Outcome
MH  - Vasodilator Agents/adverse effects/*therapeutic use
EDAT- 2006/11/03 09:00
MHDA- 2007/05/17 09:00
CRDT- 2006/11/03 09:00
PHST- 2006/11/03 09:00 [pubmed]
PHST- 2007/05/17 09:00 [medline]
PHST- 2006/11/03 09:00 [entrez]
AID - 09031936.00081706 [pii]
AID - 10.1183/09031936.00081706 [doi]
PST - ppublish
SO  - Eur Respir J. 2007 Mar;29(3):469-75. doi: 10.1183/09031936.00081706. Epub 2006 
      Nov 1.