Research and Development of a Second-Generation Process for Bosentan, an Endothelin Receptor Antagonist

2002 ◽  
Vol 6 (2) ◽  
pp. 120-124 ◽  
Author(s):  
Peter J. Harrington ◽  
Hiralal N. Khatri ◽  
Brad S. DeHoff ◽  
Martin R. Guinn ◽  
Mark A. Boehler ◽  
...  
Keyword(s):  
Research And Development ◽  
Receptor Antagonist ◽  
Second Generation ◽  
Endothelin Receptor Antagonist ◽  
Generation Process ◽  
Endothelin Receptor

scholarly journals Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

2021 ◽  
pp. 1-9
Author(s):  
Lars Søndergaard ◽  
Jamil Aboulhosn ◽  
Yves d’Udekem ◽  
Céline Faure ◽  
Wayne J Franklin ◽  
...  
Keyword(s):  
Creatinine Clearance ◽  
Receptor Antagonist ◽  
Functional Class ◽  
Fontan Circulation ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Converting Enzyme Inhibitors ◽  
Nyha Functional Class ◽  
Lateral Tunnel ◽  
Adolescents And Adults

Abstract The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with “stable” Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial’s eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in “stable” patients.

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