Pulmonary arterial hypertension in children

A Widlitz, RJ Barst - European Respiratory Journal, 2003 - Eur Respiratory Soc
For physicians to admit that a group of patients remains for whom no cure is available in
modern medicine is intellectually unsatisfying. Pulmonary arterial hypertension is a rare …

Pulmonary arterial hypertension in children

EB Rosenzweig, AC Widlitz, RJ Barst - Pediatric pulmonology, 2004 - Wiley Online Library
Pulmonary arterial hypertension is a serious progressive condition with a poor prognosis if
not identified and treated early. Because the symptoms are nonspecific and the physical …

Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension

RJ Barst, D Ivy, J Dingemanse, A Widlitz… - Clinical …, 2003 - Wiley Online Library
Background Bosentan, a dual endothelin‐receptor antagonist, is registered for the treatment
of pulmonary arterial hypertension. Little is known about the effects of bosentan in children. …

BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease

…, WPK Wong, E Yen, A Widlitz… - European …, 2004 - Eur Respiratory Soc
The aim of the present study was to determine if patients with both pulmonary arterial
hypertension (PAH), due to pulmonary vascular obstructive disease, and congenital heart defects (…

Effects of long-term bosentan in children with pulmonary arterial hypertension

EB Rosenzweig, DD Ivy, A Widlitz, A Doran… - Journal of the American …, 2005 - jacc.org
Objectives : This study investigated the long-term outcome of children with pulmonary arterial
hypertension (PAH) treated with bosentan therapy, with or without concomitant prostanoid …

New predictors of outcome in idiopathic pulmonary arterial hypertension

…, RP Garofano, RL Goldsmith, AC Widlitz… - The American journal of …, 2005 - Elsevier
Idiopathic pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis.
New therapies have improved the outcome of this condition; accordingly, the factors that …

Transition from intravenous epoprostenol to intravenous treprostinil in pulmonary hypertension

…, VV McLaughlin, A Krichman, AC Widlitz… - American journal of …, 2005 - atsjournals.org
Rationale: Intravenous epoprostenol improves exercise capacity and survival in patients with
pulmonary arterial hypertension. The prostacyclin analog treprostinil is also efficacious by …

Outcomes in children with idiopathic pulmonary arterial hypertension

D Yung, AC Widlitz, EB Rosenzweig, D Kerstein… - Circulation, 2004 - Am Heart Assoc
Background— Treatment for idiopathic pulmonary arterial hypertension in children includes
calcium channel blockade (CCB) for acute responders with vasodilator testing and chronic …

Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial

…, VV McLaughlin, RL Benza, AC Widlitz… - Chest, 2006 - Elsevier
Background: Pulmonary arterial hypertension (PAH) is a life-threatening disease for which
both continuous IV epoprostenol and continuous subcutaneous treprostinil have proven …

Clinical efficacy of sitaxsentan, an endothelin-A receptor antagonist, in patients with pulmonary arterial hypertension: open-label pilot study

RJ Barst, S Rich, A Widlitz, EM Horn, V McLaughlin… - Chest, 2002 - Elsevier
Study objectives To evaluate the safety and efficacy of sitaxsentan, an endothelin-A receptor
antagonist, in a 12-week, open-label trial of patients with pulmonary arterial hypertension (…