Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2010 Jun 4;26(3):206-218.

A New Era in Medical Management of Severe Pediatric Pulmonary Arterial Hypertension

Dunbar Ivy  1 , Ben T Saji
Affiliations

A New Era in Medical Management of Severe Pediatric Pulmonary Arterial Hypertension

Dunbar Ivy et al. Nihon Shoni Junkanki Gakkai Zasshi. .

Abstract

Pulmonary arterial hypertension (PAH) is a life-threatening disease whose prognosis has changed dramatically over the past decade since the introduction of new therapeutic agents as well as the off-label application of adult pulmonary hypertension specific therapies to children. Nevertheless, PAH still has no cure and the aim of treatment is to prolong survival by improving quality of life, symptoms, exercise capacity and hemodynamics. The selection of appropriate therapies for PH is complex and must be carefully chosen according to the etiology and pulmonary vasoreactivity. As insight advances into mechanisms responsible for the development of PAH, the introduction of novel therapeutic agents will hopefully further improve the outcome of this incurable disease.

PubMed Disclaimer

References

    1. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–S54. - PubMed
    1. van Loon RL, Roofthooft MT, van Osch-Gevers M, et al. Clinical characterization of pediatric pulmonary hypertension: complex presentation and diagnosis. J Pediatr. 2009;155:176–182. e1. - PubMed
    1. Rosenzweig EB, Barst RJ. Pulmonary arterial hypertension in children: a medical update. Indian J Pediatr. 2009;76:77–81. - PubMed
    1. Beghetti M, Tissot C. Pulmonary arterial hypertension in congenital heart diseases. Semin Respir Crit Care Med. 2009;30:421–428. - PubMed
    1. Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001–2006. Heart. 2009;95:312–317. - PubMed