SIR, We read with interest the recent reports describing the promising results of studies exploring the role of the dual endothelin receptor antagonist bosentan in pulmonary arterial hypertension (PAH) w1, 2x. We report the case of a patient with systemic sclerosis associated with PAH and ischaemic digital ulcerations who responded successfully to bosentan. A 50-yr-old male dentist presented in...

Treatment of pulmonary arterial hypertension with the endothelin receptor antagonist bosentan has been associated with transient increases in liver transaminases. Mechanistically, bosentan inhibits the bile salt export pump (BSEP) leading to an intrahepatic accumulation of cytotoxic bile salts, which eventually results in hepatocellular damage. BSEP inhibition by bosentan is amplified by its ac...

The aim of the present study was to evaluate a 5-yr experience of bosentan in children with pulmonary arterial hypertension (PAH). A retrospective, observational study was made of children in the UK Pulmonary Hypertension Service for Children (Great Ormond Street Hospital for Children, London, UK) who were given bosentan as monotherapy or in combination, from February 2002 to May 2008 and follo...

In the present study, the role of endothelin-1 (ET-1) on alterations of hepatic and renal metallothionein (MT) and trace metals (Zn, Cu, and Fe) were investigated in streptozotocin (STZ)-induced diabetic rats. Diabetic rats, age- and sex-matched controls, as well as control and diabetic animals on a dual ETA/ETB receptor blocker, bosentan, were investigated after 6 months of follow-up. MT was m...

A 13 months male child presented with progressive dyspnea of three months and recent cyanosis. Cardio-respiratory examination revealed tachypnea, cyanosis, left parasternal heave and loud P2. Echocardiography revealed thrombus in pulmonary artery and severe pulmonary arterial hypertension. Tests for throbmophilia demonstrated protein S deficiency. Treatment included anticoagulants, sildenafil, ...

Annals Academy of Medicine Dear Editor, Bosentan is an endothelin-1 (ET-1) receptor antagonist approved for the treatment of pulmonary artery hypertension (PAH). Although fl ushing is a documented side effect of bosentan, the diagnosis can mimic other drug rashes and medical conditions where facial telangiectasis and/or fl ushing are prominent presentation. We present a 76-yearold woman complai...

Prostacyclin improves symptoms, exercise tolerance, and survival in patients with pulmonary arterial hypertension. However, the difficulty of administration (whether intravenous, subcutaneous, or by inhalation) often causes side effects that can reduce the patient's quality of life and which may sometimes be serious. Bosentan, an orally active endothelin receptor antagonist, improves functional...

Pulmonary arterial hypertension (PAH) is often treated with endothelin (ET) receptor blockade or phosphodiesterase-5 (PDE5) inhibition. Little is known about the specific effects on right ventricular (RV) function and metabolism. We determined the effects of single and combination treatment with Bosentan [an ET type A (ET(A))/type B (ET(B)) receptor blocker] and Sildenafil (a PDE5 inhibitor) on...