Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome

Giorgio Serino, Marco Guazzi, Angelo Micheletti, Carlo Lombardi, Rossella Danesi, Diana Negura, Mario Carminati, Massimo Chessa

Research output: Contribution to journalArticle

Abstract

This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.

Original languageEnglish
Pages (from-to)29-34
Number of pages6
JournalClinical Medicine Insights: Cardiology
Volume7
DOIs
Publication statusPublished - 2013

Fingerprint

Eisenmenger Complex
Down Syndrome
Exercise
Doppler Echocardiography
Pulmonary Hypertension
Therapeutics
Drug-Related Side Effects and Adverse Reactions
Hospitalization
Maintenance
bosentan
Liver

Keywords

  • Bosentan
  • Congenital heart disease
  • Down syndrome
  • Eisenmenger syndrome
  • Endothelin receptor antagonist
  • Pulmonary arterial hypertension

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome. / Serino, Giorgio; Guazzi, Marco; Micheletti, Angelo; Lombardi, Carlo; Danesi, Rossella; Negura, Diana; Carminati, Mario; Chessa, Massimo.

In: Clinical Medicine Insights: Cardiology, Vol. 7, 2013, p. 29-34.

Research output: Contribution to journalArticle

@article{d62e0b708f4245eeb8476a20ba17a473,
title = "Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome",
abstract = "This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.",
keywords = "Bosentan, Congenital heart disease, Down syndrome, Eisenmenger syndrome, Endothelin receptor antagonist, Pulmonary arterial hypertension",
author = "Giorgio Serino and Marco Guazzi and Angelo Micheletti and Carlo Lombardi and Rossella Danesi and Diana Negura and Mario Carminati and Massimo Chessa",
year = "2013",
doi = "10.4137/CMC.S10237",
language = "English",
volume = "7",
pages = "29--34",
journal = "Clinical Medicine Insights: Cardiology",
issn = "1179-5468",
publisher = "Libertas Academica Ltd.",

}

TY - JOUR

T1 - Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome

AU - Serino, Giorgio

AU - Guazzi, Marco

AU - Micheletti, Angelo

AU - Lombardi, Carlo

AU - Danesi, Rossella

AU - Negura, Diana

AU - Carminati, Mario

AU - Chessa, Massimo

PY - 2013

Y1 - 2013

N2 - This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.

AB - This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.

KW - Bosentan

KW - Congenital heart disease

KW - Down syndrome

KW - Eisenmenger syndrome

KW - Endothelin receptor antagonist

KW - Pulmonary arterial hypertension

UR - http://www.scopus.com/inward/record.url?scp=84873895415&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84873895415&partnerID=8YFLogxK

U2 - 10.4137/CMC.S10237

DO - 10.4137/CMC.S10237

M3 - Article

C2 - 23440179

AN - SCOPUS:84873895415

VL - 7

SP - 29

EP - 34

JO - Clinical Medicine Insights: Cardiology

JF - Clinical Medicine Insights: Cardiology

SN - 1179-5468

ER -