Document Detail

Etiology, diagnosis, and pharmacologic treatment of pediatric pulmonary hypertension.
MedLine Citation:
PMID:  19301933     Owner:  NLM     Status:  MEDLINE    
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.
Robert Tulloh
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Paediatric drugs     Volume:  11     ISSN:  1174-5878     ISO Abbreviation:  Paediatr Drugs     Publication Date:  2009  
Date Detail:
Created Date:  2009-03-23     Completed Date:  2009-08-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883685     Medline TA:  Paediatr Drugs     Country:  New Zealand    
Other Details:
Languages:  eng     Pagination:  115-28     Citation Subset:  IM    
Department of Congenital Heart Disease, Bristol Royal Hospital for Children and Bristol Royal Infirmary, Bristol, England.
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MeSH Terms
Administration, Oral
Epoprostenol / pharmacology,  therapeutic use
Heart Catheterization
Hypertension, Pulmonary* / drug therapy,  etiology,  therapy
Infusions, Intravenous
Oxygen / pharmacology,  therapeutic use
Oxygen Inhalation Therapy
Phosphodiesterase Inhibitors / pharmacology,  therapeutic use
Piperazines / pharmacology,  therapeutic use
Prostaglandins / pharmacology,  therapeutic use
Purines / pharmacology,  therapeutic use
Receptors, Endothelin / antagonists & inhibitors
Sulfones / pharmacology,  therapeutic use
Reg. No./Substance:
0/Phosphodiesterase Inhibitors; 0/Piperazines; 0/Prostaglandins; 0/Purines; 0/Receptors, Endothelin; 0/Sulfones; 139755-83-2/sildenafil; 35121-78-9/Epoprostenol; 7782-44-7/Oxygen

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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