Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is
associated with significant morbidity and mortality. In the majority of pediatric patients, PH is …

The Development and Pathology working group was charged with reviewing the present
knowledge, gaps in understanding, and areas for further studies in a broad range of themes …

To date, randomized controlled clinical trials performed in pulmonary arterial hypertension
(PAH) have been relatively short-term studies involving mainly patients with advanced …

Fifty patients with congenital heart disease, ages 2 days-30 years (median 12 months) at
cardiac surgery, underwent lung biopsy to assess pulmonary vascular disease (PVD) …

Objective: The 6-minute walk test (6MWT) is an established measure of exercise capacity in
adults and children with chronic cardiac or respiratory disease. Despite its widespread use …

Current classifications of pulmonary hypertension have contributed a great deal to our
understanding of pulmonary vascular disease, facilitated drug trials, and improved our …

Objective: A retrospective study of the UK Pulmonary Hypertension Service for Children for
the first 5-year period of its existence. Design and patients: Records of 216 children with …

Background—Pulmonary arterial hypertension (PAH) is a potentially fatal vasculopathy that
can develop at any age. Adult-onset disease has previously been associated with mutations …

Objective To clarify the clinical characteristics and epidemiology of idiopathic pulmonary
arterial hypertension (IPAH) in childhood, a rare condition with a bad prognosis, poorly …

Rationale: Prostacyclin analogs, used to treat idiopathic pulmonary arterial hypertension
(IPAH), are assumed to work through prostacyclin (IP) receptors linked to cyclic AMP (cAMP) …