Document Detail

Clinical practice: pulmonary hypertension in children.
MedLine Citation:
PMID:  19148677     Owner:  NLM     Status:  MEDLINE    
INTRODUCTION: Pulmonary arterial hypertension is a rare disorder in childhood, the two most common types being idiopathic pulmonary arterial hypertension and pulmonary hypertension associated with congenital left-to-right shunt lesions, together accounting for almost 90% of cases.
DISCUSSION: The clinical presentation of idiopathic pulmonary arterial hypertension (familial and non familial) is essentially non-specific and varies with age. Pulmonary vasoreactivity testing identifies responders and non-responders. Responders are treated with calcium channel blockers and have a better prognosis. Non-responders have a very limited survival beyond diagnosis if not treated with more selective pulmonary arterial vasodilators. Prostacyclin, endothelin receptor antagonists and phosphodiesterase-5 inhibitors improve haemodynamics, functional class and exercise tolerance and delay deterioration. Patients with congenital left-to-right shunts and irreversible pulmonary arterial hypertension leading to Eisenmenger's syndrome have multiple organ disease. Despite a very pronounced exercise intolerance, their clinical course is rather stable with survival up to 40-60 years, depending on the complexity of their underlying cardiac defect. Treatment is based on general measures along with the same three types of selective pulmonary vasodilators as in idiopathic pulmonary arterial disease. Improvement in haemodynamics, functional class and exercise tolerance are comparable for both patient groups.
CONCLUSION: Pulmonary hypertension in children is idiopathic or associated with congenital heart disease in the majority of patients. Treatment with new selective pulmonary vasodilators offers haemodynamic and functional improvement.
Daniel De Wolf
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Publication Detail:
Type:  Journal Article     Date:  2009-01-16
Journal Detail:
Title:  European journal of pediatrics     Volume:  168     ISSN:  1432-1076     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-03-18     Completed Date:  2009-07-27     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  515-22     Citation Subset:  IM    
Pediatric cardiology UZ Gent, De Pintelaan 185, 9000 Gent, Belgium.
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MeSH Terms
Activin Receptors, Type II / genetics
Antigens, CD / genetics
Antihypertensive Agents / therapeutic use*
Bone Morphogenetic Protein Receptors, Type II / genetics
Calcium Channel Blockers / therapeutic use
Heart Defects, Congenital / diagnosis,  drug therapy*,  epidemiology*
Hypertension, Pulmonary / drug therapy*,  epidemiology*,  genetics
Piperazines / therapeutic use*
Point Mutation / genetics
Purines / therapeutic use
Receptors, Cell Surface / genetics
Sulfonamides / therapeutic use*
Sulfones / therapeutic use*
Vasodilator Agents / therapeutic use*
Reg. No./Substance:
0/Antigens, CD; 0/Antihypertensive Agents; 0/Calcium Channel Blockers; 0/ENG protein, human; 0/Piperazines; 0/Purines; 0/Receptors, Cell Surface; 0/Sulfonamides; 0/Sulfones; 0/Vasodilator Agents; 3M7OB98Y7H/sildenafil; EC protein, human; EC Receptors, Type II; EC protein, human; EC Morphogenetic Protein Receptors, Type II; Q326023R30/bosentan

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

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