PMID- 20304507
OWN - NLM
STAT- MEDLINE
DCOM- 20111019
LR  - 20151119
IS  - 1874-1754 (Electronic)
IS  - 0167-5273 (Linking)
VI  - 149
IP  - 3
DP  - 2011 Jun 16
TI  - Quality of life and functional capacity can be improved in patients with 
      Eisenmenger syndrome with oral sildenafil therapy.
PG  - 372-6
LID - 10.1016/j.ijcard.2010.02.020 [doi]
AB  - BACKGROUND: Patients with Eisenmenger syndrome (ES) have a decreased exercise 
      capacity and poor quality of life (QoL). While patients may survive to middle 
      adulthood, the burden of disease is disabling. Sildenafil seems to improve 
      exercise tolerance and hemodynamics, but there is no data to date on its impact 
      on QoL. METHODS: Eisenmenger patients in New York Heart Association (NYHA) class 
      III were recruited in a prospective study of efficacy and safety of oral 
      sildenafil. The QoL endpoint was assessed using a disease-specific questionnaire 
      (CAMPHOR). Exercise capacity was assessed by means of six minute walk test 
      (6MWT). All patients underwent comprehensive assessment at baseline and after 
      3months of treatment. RESULTS: Twelve patients (mean age was 34.3+/-10.2, 83% 
      female) with various cardiac anatomies were recruited. No major adverse events 
      during the follow-up or significant drop in resting oxygen saturation were 
      recorded. After 3months of oral sildenafil therapy, all patients improved to NYHA 
      II with a concomitant improvement in 6MWT distance (347.3+/-80.7 to 392.5+/-82.0m, 
      p=0.002). All components of the CAMPHOR score, relating to symptoms, activity and 
      QoL, improved significantly resulting in substantial improvement in the total 
      CAMPHOR score (27.6+/-10.5 to 15.8+/-10.4, p=0.002). CONCLUSIONS: Three months of 
      sildenafil therapy in adults with ES was well tolerated and associated with 
      significant improvement in the QoL CAMPHOR questionnaire and in NYHA class and 
      exercise capacity. Larger studies are warranted to assess long term efficacy of 
      oral sildenafil and potential impact on survival.
CI  - Copyright (c) 2010 Elsevier Ireland Ltd. All rights reserved.
FAU - Tay, Edgar L W
AU  - Tay EL
AD  - Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal 
      Brompton Hospital, London, UK.
FAU - Papaphylactou, Maria
AU  - Papaphylactou M
FAU - Diller, Gerhard Paul
AU  - Diller GP
FAU - Alonso-Gonzalez, Rafael
AU  - Alonso-Gonzalez R
FAU - Inuzuka, Ryo
AU  - Inuzuka R
FAU - Giannakoulas, Georgios
AU  - Giannakoulas G
FAU - Harries, Carl
AU  - Harries C
FAU - Wort, Stephen John
AU  - Wort SJ
FAU - Swan, Lorna
AU  - Swan L
FAU - Dimopoulos, Konstantinos
AU  - Dimopoulos K
FAU - Gatzoulis, Michael A
AU  - Gatzoulis MA
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20100320
PL  - Netherlands
TA  - Int J Cardiol
JT  - International journal of cardiology
JID - 8200291
RN  - 0 (Piperazines)
RN  - 0 (Purines)
RN  - 0 (Sulfones)
RN  - 0 (Vasodilator Agents)
RN  - BW9B0ZE037 (Sildenafil Citrate)
SB  - IM
MH  - Administration, Oral
MH  - Adult
MH  - Eisenmenger Complex/*drug therapy/*physiopathology
MH  - Exercise Tolerance/*drug effects
MH  - Female
MH  - Humans
MH  - Male
MH  - Piperazines/*administration & dosage/adverse effects
MH  - Prospective Studies
MH  - Purines/administration & dosage/adverse effects
MH  - *Quality of Life
MH  - Sildenafil Citrate
MH  - Sulfones/*administration & dosage/adverse effects
MH  - Surveys and Questionnaires
MH  - Treatment Outcome
MH  - Vasodilator Agents/*administration & dosage/adverse effects
MH  - Young Adult
EDAT- 2010/03/23 06:00
MHDA- 2011/10/20 06:00
CRDT- 2010/03/23 06:00
PHST- 2009/07/23 00:00 [received]
PHST- 2009/10/20 00:00 [revised]
PHST- 2010/02/14 00:00 [accepted]
PHST- 2010/03/23 06:00 [entrez]
PHST- 2010/03/23 06:00 [pubmed]
PHST- 2011/10/20 06:00 [medline]
AID - S0167-5273(10)00087-2 [pii]
AID - 10.1016/j.ijcard.2010.02.020 [doi]
PST - ppublish
SO  - Int J Cardiol. 2011 Jun 16;149(3):372-6. doi: 10.1016/j.ijcard.2010.02.020. Epub 
      2010 Mar 20.