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The management of pulmonary hypertension in children.

Haworth SG - Arch. Dis. Child. (2008)

Bottom Line: The management of the condition has changed dramatically in the past 5 years with the introduction of new medicines.However, diagnosis, investigation and choice of therapy remain a challenge.This service, based at Great Ormond Street Hospital for Children, provides advice, expertise and infrastructure support for the most severely affected patients, particularly those with idiopathic pulmonary arterial hypertension for whom chronic intravenous prostacyclin remains the most effective medication.

View Article: PubMed Central - PubMed

Affiliation: Great Ormond Street Hospital for Children, London WC1N 3 JH, UK. s.haworth@ich.ucl.ac.uk

ABSTRACT
Pulmonary hypertension is relatively common in children and has many causes. The management of the condition has changed dramatically in the past 5 years with the introduction of new medicines. However, diagnosis, investigation and choice of therapy remain a challenge. In 2002 the United Kingdom Pulmonary Hypertension Service for Children was established and this has become the mainstay of management in this country. This service, based at Great Ormond Street Hospital for Children, provides advice, expertise and infrastructure support for the most severely affected patients, particularly those with idiopathic pulmonary arterial hypertension for whom chronic intravenous prostacyclin remains the most effective medication. New medicines are being developed which, rather than focussing on dilating a diseased pulmonary vascular bed, aim to structurally remodel the pulmonary vasculature towards normal.

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Related in: MedlinePlus

Investigating pulmonary hypertension: diagnostic strategy. CXR, chest x ray; PH, pulmonary hypertension; V/Q, ventilation/perfusion.
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adc-93-07-0620-f01: Investigating pulmonary hypertension: diagnostic strategy. CXR, chest x ray; PH, pulmonary hypertension; V/Q, ventilation/perfusion.

Mentions: In 1998 the only drugs available to treat pulmonary hypertension were calcium channel antagonists and intravenous epoprostenol, although epoprostenol was only shown to be efficacious in children with IPAH in 1999.5 Now there are effective endothelial receptor antagonists, phosphodiesterase inhibitors and prostacyclin analogues, the first two groups of drugs being effective orally.


The management of pulmonary hypertension in children.

Haworth SG - Arch. Dis. Child. (2008)

Investigating pulmonary hypertension: diagnostic strategy. CXR, chest x ray; PH, pulmonary hypertension; V/Q, ventilation/perfusion.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2532955&req=5

adc-93-07-0620-f01: Investigating pulmonary hypertension: diagnostic strategy. CXR, chest x ray; PH, pulmonary hypertension; V/Q, ventilation/perfusion.
Mentions: In 1998 the only drugs available to treat pulmonary hypertension were calcium channel antagonists and intravenous epoprostenol, although epoprostenol was only shown to be efficacious in children with IPAH in 1999.5 Now there are effective endothelial receptor antagonists, phosphodiesterase inhibitors and prostacyclin analogues, the first two groups of drugs being effective orally.

Bottom Line: The management of the condition has changed dramatically in the past 5 years with the introduction of new medicines.However, diagnosis, investigation and choice of therapy remain a challenge.This service, based at Great Ormond Street Hospital for Children, provides advice, expertise and infrastructure support for the most severely affected patients, particularly those with idiopathic pulmonary arterial hypertension for whom chronic intravenous prostacyclin remains the most effective medication.

View Article: PubMed Central - PubMed

Affiliation: Great Ormond Street Hospital for Children, London WC1N 3 JH, UK. s.haworth@ich.ucl.ac.uk

ABSTRACT
Pulmonary hypertension is relatively common in children and has many causes. The management of the condition has changed dramatically in the past 5 years with the introduction of new medicines. However, diagnosis, investigation and choice of therapy remain a challenge. In 2002 the United Kingdom Pulmonary Hypertension Service for Children was established and this has become the mainstay of management in this country. This service, based at Great Ormond Street Hospital for Children, provides advice, expertise and infrastructure support for the most severely affected patients, particularly those with idiopathic pulmonary arterial hypertension for whom chronic intravenous prostacyclin remains the most effective medication. New medicines are being developed which, rather than focussing on dilating a diseased pulmonary vascular bed, aim to structurally remodel the pulmonary vasculature towards normal.

Show MeSH
Related in: MedlinePlus