Document Detail

Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy.
MedLine Citation:
PMID:  20668108     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Vasoreactivity testing is recommended in the management of pulmonary arterial hypertension (PAH), but its clinical relevance in congenital heart disease (CHD)-associated PAH has not been established.
OBJECTIVE: To determine whether residual pulmonary vascular responsiveness to intravenous. epoprostenol is predictive of clinical outcome in patients with CHD-PAH and Eisenmenger syndrome.
METHODS AND RESULTS: A diagnostic right heart catheterisation with reversibility testing using epoprostenol infusion was performed in 38 consecutive patients with CHD-PAH and Eisenmenger syndrome. Patients were treated with bosentan and were assessed every 3 months. Clinical worsening was defined as death from any cause, heart-lung or lung transplantation (or on the waiting list for this procedure), hospitalisation for PAH, or symptom exacerbation defined as a > or =20% decrease in the 6 min walking distance on two consecutive tests, an increase in WHO functional class, or worsening right heart failure. The mean follow-up was 33+/-17 months. Sixteen patients showed clinical worsening. Although they did not differ from the other patients in their baseline exercise capacity, haemodynamic characteristics and underlying CHD, pulmonary vascular resistance index (PVRi) was less reversible (DeltaPVRi 29+/-21 vs 52+/-14%, p=0.0003). At univariate analysis, systemic vascular resistance, PVRi and DeltaPVRi were significant predictors of clinical worsening. At multivariate Cox proportional hazards regression model, DeltaPVRi was found to be the only independent predictor of clinical worsening (HR=0.973, 95% CI 0.95 to 0.99; p=0.01). DeltaPVRi > or =25% had a positive and negative predictive value for clinical worsening of 100% and 75.9%, respectively.
CONCLUSION: Pulmonary vasoreactivity is a significant predictor of clinical worsening in patients with CHD-PAH.
Michele D'Alto; Emanuele Romeo; Paola Argiento; Giuseppe Santoro; Berardo Sarubbi; Giampiero Gaio; Christian Mélot; Maria Giovanna Russo; Robert Naeije; Raffaele Calabrò
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Publication Detail:
Type:  Journal Article     Date:  2010-07-28
Journal Detail:
Title:  Heart (British Cardiac Society)     Volume:  96     ISSN:  1468-201X     ISO Abbreviation:  Heart     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-09-03     Completed Date:  2010-10-28     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  9602087     Medline TA:  Heart     Country:  England    
Other Details:
Languages:  eng     Pagination:  1475-9     Citation Subset:  AIM; IM    
Second University of Naples, A.O. V. Monaldi, Naples, Italy.
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MeSH Terms
Antihypertensive Agents / diagnostic use,  therapeutic use*
Cardiac Catheterization / methods
Eisenmenger Complex / drug therapy*,  physiopathology
Epidemiologic Methods
Epoprostenol / diagnostic use
Exercise Tolerance / physiology
Hemodynamics / physiology
Hypertension, Pulmonary / drug therapy*,  physiopathology
Middle Aged
Pulmonary Artery / physiopathology
Sulfonamides / therapeutic use*
Treatment Outcome
Vascular Resistance / drug effects
Young Adult
Reg. No./Substance:
0/Antihypertensive Agents; 0/Sulfonamides; 35121-78-9/Epoprostenol; Q326023R30/bosentan

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