Journal Information
Vol. 42. Issue 12.
Pages 616-620 (December 2006)
Share
Share
Download PDF
More article options
Vol. 42. Issue 12.
Pages 616-620 (December 2006)
Original Articles
Full text access
Long-term Outcomes of Treatment With Bosentan in Pulmonary Hypertension
Visits
4038
Antonio Romána,
Corresponding author
aroman@vhebron.net

Correspondence: Dr. A. Román. Servei de Pneumologia. Hospital Universitari Vall d'Hebron. Pg. Vall d'Hebron, 119-129. 08035 Barcelona. España
, Pilar Gisperta, Víctor Monfortea, Carlos Bravoa, Enric Domingob, Ferran Morella
a Servei de Pneumologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain
b Servei de Cardiologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain
Related content
Arch Bronconeumol. 2007;43:24310.1016/S1579-2129(07)60060-3
This item has received
Article information
Objective

Treatment with bosentan improves exercise capacity in patients with pulmonary hypertension. Few studies have assessed treatment with this drug over long periods. The aim was therefore to assess long-term treatment with bosentan.

Patients and methods

A group of 22 functional class III patients—18 women and 4 men, mean age, 45.5 years (range, 19-77 years)—with pulmonary hypertension were treated with bosentan between April 2002 and June 2005. Pulmonary hypertension was idiopathic in 10 patients. In the remaining patients, etiologies were associated with compensated heart failure (n=4), scleroderma (n=4), peripheral embolism (n=3), and portal hypertension (n=1). Clinical and hemodynamic variables and their changes between baseline and the end of study were analyzed.

Results

The mean duration of follow-up of the patients was 15.7 months (range, 12.6-31.8 months). Functional class improved or stabilized after 3 months of treatment in 21 (95%) and after 1 year in 14 (64%). At 3 months, the distance covered in the 6-minute walk test increased by a mean of 64.5 m, an improvement that was maintained at 6, 12, and 18 months. Treatment was interrupted in 4 patients (18%). Reasons for discontinuation were death in 2 patients, deterioration in 1 patient, and intolerance of the medication in 1 patient. Treatment was ineffective for 4 patients (18%). No patient experienced notable liver toxicity.

Conclusions

The results of this study suggest that treatment with bosentan is associated with long-term improvement in clinical variables and exercise capacity in approximately two thirds of the patients with pulmonary hypertension.

Key words:
Bosentan
Endothelin receptors
Pulmonary hypertension
Objetivo

El tratamiento con bosentán mejora la capacidad de ejercicio de los pacientes con hipertensión pulmonar. Son muy escasos los estudios que evalúan este tratamiento a largo plazo, que es el objetivo propuesto en el presente trabajo.

Pacientes y métodos

Un grupo de 22 pacientes −18 mu-jeres y 4 varones, con una edad media de 45,5 años (rango: 19-77 años)- con hipertensión pulmonar en clase funcional III recibieron tratamiento con bosentán entre abril de 2002 y junio de 2005. La hipertensión pulmonar era idiopática en 10 casos y estaba asociada a cardiopatía corregida (n = 4), escle-rodermia (n = 4), embolia periférica (n = 3) e hipertensión portal (n = 1) en los restantes. Se estudiaron los datos clínicos y hemodinámicos y los cambios que habían experimentado desde el inicio del tratamiento hasta el fin del estudio.

Resultados

El seguimiento medio de los pacientes fue de 15,7 meses (rango: 12,6-31,8). La clase funcional mejoró o se estabilizó a los 3 meses de tratamiento en 21 (95%) y al año en 14 (64%). La distancia recorrida en la prueba de la mar-cha de 6 min se incrementó a los 3 meses de tratamiento una media de 64,5 m, mejoría que se mantuvo a los 6, 12 y 18 meses. El tratamiento se interrumpió en 4 pacientes (18%): por muerte en 2 casos, por empeoramiento en uno y por in-tolerancia a la medicación en otro. En otros 4 pacientes (18%) se objetivó falta de eficacia. Ningún paciente presentó hepatotoxicidad remarcable.

Conclusiones

Los resultados del presente estudio apo-yan la idea de que el tratamiento con bosentán se asocia a una mejoría clínica y de la capacidad de ejercicio a largo plazo en aproximadamente dos terceras partes de los pacientes con hipertensión pulmonar.

Palabras clave:
Bosentán
Receptores de la endotelina
Endotelina
Hipertensión arterial pulmonar
Full text is only aviable in PDF
REFERENCES
[1]
GE D'Alonzo, RJ Barst, SM Ayres, EH Bergofsky, BH Brundage, KM Detre, et al.
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.
Ann Intern Med, 115 (1991), pp. 343-349
[2]
C Rubens, R Ewert, M Halank, R Wensel, HD Orzechowski, HP Schultheiss, et al.
Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension.
Chest, 120 (2001), pp. 1562-1569
[3]
M Bauer, HC Wilkens, F Langer, SO Schneider, H Lausberg, HJ Schafers.
Selective upregulation of endothelin B receptor gene expression in severe pulmonary hypertension.
Circulation, 105 (2002), pp. 1034-1036
[4]
N Galie, AL Hinderliter, A Torbicki, T Fourme, G Simonneau, T Pulido, et al.
Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension.
J Am Coll Cardiol, 41 (2003), pp. 1380-1386
[5]
RN Channick, G Simonneau, O Sitbon, IM Robbins, A Frost, VF Tapson, et al.
Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study.
Lancet, 358 (2001), pp. 1119-1123
[6]
LJ Rubin, DB Badesch, RJ Barst, N Galie, CM Black, A Keogh, et al.
Bosentan therapy for pulmonary arterial hypertension.
N Engl J Med, 346 (2002), pp. 896-903
[7]
O Sitbon, VV McLaughlin, DB Badesch, RJ Barst, C Black, N Galie, et al.
Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol.
Thorax, 60 (2005), pp. 1025-1030
[8]
HF van Stel, JM Bogaard, LHM Rijssenbeek-Nouwens, VT Colland.
Multivariable assessment of the 6-min walking test in patients with chronic obstructive pulmonary disease.
Am J Respir Crit Care Med, 163 (2001), pp. 1567-1571
[9]
S Provencher, O Sitbon, M Humbert, S Cabrol, X Jais, G Simonneau.
Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension.
Eur Heart J, 27 (2006), pp. 589-595
[10]
VV McLaughlin, O Sitbon, DB Badesch, RJ Barst, C Black, N Galie, et al.
Survival with first-line bosentan in patients with primary pulmonary hypertension.
Eur Respir J, 25 (2005), pp. 942
[11]
O Sitbon, DB Basesch, RN Channick, A Frost, IM Robbins, G Simonneau, et al.
Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension–a 1-year follow-up study.
Chest, 124 (2003), pp. 247-254
Copyright © 2006. Sociedad Española de Neumología y Cirugía Torácica (SEPAR)
Archivos de Bronconeumología
Article options
Tools

Are you a health professional able to prescribe or dispense drugs?