title: Etiology, diagnosis, and pharmacologic treatment of pediatric pulmonary hypertension.
abstract: Major advances have been made in the understanding and treatment of pulmonary hypertension in the last few years. Without treatment (medication) for idiopathic pulmonary arterial hypertension, which is a rare and potentially fatal condition, the survival time is only about 3 years after diagnosis. However, if pulmonary hypertension is secondary to other causes such as congenital heart disease, it is possible to survive for 30 years or more without treatment. The condition can affect children at any age, from fetal life to adulthood. Patients with pulmonary hypertension can present to the respiratory pediatrician with unresponsive asthma, to the neurologist with faints, or to the general pediatrician with failure to thrive. Over the last few years there have been significant developments in the available therapy for managing this complicated disease. There is now a generally recognized ladder of long-term therapy for chronic pulmonary hypertension. Treatment can start with oxygen at home at night or even during the day. Next is the use of oral phosphodiesterase inhibitors, mostly type V, such as sildenafil, which enhance endogenous nitric oxide. More potent are the endothelin receptor antagonists and the most potent are the prostanoids, especially epoprostenol, which is given by constant intravenous infusion. In addition to interventional catheterization with atrial septostomy, these agents have improved the prognostic outlook. This article reviews the current knowledge about the etiology, investigation, and treatment of children with pulmonary hypertension in the clinical setting.
authors.affiliation: Department of Congenital Heart Disease, Bristol Royal Hospital for Children and Bristol Royal Infirmary, Bristol, England. Robert.Tulloh@ubht.nhs.uk
authors.forename: Robert
authors.initials: R
authors.lastname: Tulloh
mesh_terms: D000284:Administration, Oral; D006328:Cardiac Catheterization; D002648:Child; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D007262:Infusions, Intravenous; D010100:Oxygen / Q000494:pharmacology / Q000627:therapeutic use; D010102:Oxygen Inhalation Therapy; D010726:Phosphodiesterase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use; D011453:Prostaglandins / Q000494:pharmacology / Q000627:therapeutic use; D011687:Purines / Q000494:pharmacology / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011453:Prostaglandins; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D010100:Oxygen