An update on the diagnosis and treatment of pediatric pulmonary hypertension.

Introduction: Pulmonary hypertension (PH) is a heterogeneous disease that mainly affects the pulmonary arterioles, leading to significant morbidity and mortality. Pulmonary hypertension in children from birth to adolescence presents important differences from that of adults. The majority of pediatric pulmonary arterial hypertension (PAH) cases are idiopathic or associated with congenital heart disease.

Atrial natriuretic peptide concentrations in children with pulmonary hypertension: correlation with hemodynamic measurements.

To define the relation between atrial natriuretic peptide (ANP) and hemodynamic parameters in children with pulmonary artery hypertension, we measured the ANP concentrations of the femoral vein, right atrium, pulmonary artery, left atrium and left ventricle, or femoral artery in 32 patients during right or left heart catheterization. There is a strong correlation among the ANP levels obtained from different locations (p < 0.001), and these ANP levels are positively correlated with pulmonary arterial pressures and pulmonary resistance.

Diffuse arterial aneurysms in a case of Ehlers-Danlos syndrome--a case report.

A three-year-old boy with the diagnosis of Ehlers-Danlos syndrome (EDS) with persistent ductus arteriosus and multiple diffuse arterial aneurysms is presented. The case is classified as "EDS type unknown" because the clinical features and the inheritance pattern differ from the types described previously. It is stressed that the diagnosis of the disease is important for genetic counseling and surgical intervention.