Pediatric Pulmonary Hypertension

1. Hari Rajagopal, MD* 2. Ruchika Karnik, MD* 3. Raj Sahulee, DO* 1. *Icahn School of Medicine at Mount Sinai, New York, NY. 1. 1. Ivy DD, 2. Abman SH, 3. Barst RJ, 4. et al Pediatric Pulmonary Hypertension. Ivy DD, Abman SH, Barst RJ, et al. J Am Coll Cardiol. 2013;62(25 suppl):D117–D126. [OpenUrl][1][CrossRef][2][PubMed][3][Web of Science][4] 2. 1. Vorhies EE, 2. Ivy DD Drug Treatment of Pulmonary Hypertension in Children. Vorhies EE, Ivy DD Paediatr Drugs. 2014;16(1):43–65. [OpenUrl][5][CrossRef][6][PubMed][7] 3. 1. Barst RJ, 2. Ertel SI, 3. Beghetti M, 4. Ivy DD Pulmonary Arterial Hypertension: A Comparison Between Children and Adults. Barst RJ, Ertel SI, Beghetti M, Ivy DD Eur Respir J. 2011;37(3):665–677. [OpenUrl][8][Abstract/FREE Full Text][9] Pulmonary hypertension (PH) is a rare disease that can present in newborns, infants, and children and is associated with considerable morbidity and mortality. The etiology and presentation of PH varies substantially between children and adults. Unfortunately, the lack of sufficient controlled studies in children makes PH management challenging, with most therapeutic strategies based on extrapolation from adult studies and expert consensus. PH is defined similarly in children and adults as a mean pulmonary artery pressure of 25 mm Hg or more at rest. The subset of such patients who have a normal pulmonary artery wedge pressure of less than 15 mm Hg are subclassified as having pulmonary arterial hypertension (PAH). PH is further subclassified according to its underlying cause, whether left heart disease, lung disease, thromboembolic disease, or a variety of other diseases. The most recent classification for PH was modified during the Pediatric Task Force of the 5th World Symposium on Pulmonary Hypertension in Nice, France. Although the exact incidence and prevalence of PH are not known, European registries have estimated the incidence to average 63.7 cases per 1 million children when including transient cases. These investigations suggest that the most common causes of transient PH in children are persistent pulmonary hypertension of the newborn and congenital heart disease (CHD). In contrast, the most common causes of persistent PH in children are idiopathic PAH and PH associated with CHD. The natural history of PH in children varies by cause, with PAH having a substantially poorer prognosis than PH with CHD. PH is characterized by a variety of vascular abnormalities, including intimal hyperplasia, medial hypertrophy, thrombosis in situ, and … [1]: {openurl}?query=rft.jtitle%253DJ%2BAm%2BColl%2BCardiol%26rft.volume%253D62%26rft.spage%253DD117%26rft_id%253Dinfo%253Adoi%252F10.1016%252Fj.jacc.2013.10.028%26rft_id%253Dinfo%253Apmid%252F24355636%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [2]: /lookup/external-ref?access_num=10.1016/j.jacc.2013.10.028&link_type=DOI [3]: /lookup/external-ref?access_num=24355636&link_type=MED&atom=%2Fpedsinreview%2F37%2F3%2F129.atom [4]: /lookup/external-ref?access_num=000329459400014&link_type=ISI [5]: {openurl}?query=rft.jtitle%253DPaediatr%2BDrugs%26rft.volume%253D16%26rft.spage%253D43%26rft_id%253Dinfo%253Adoi%252F10.1007%252Fs40272-013-0052-2%26rft_id%253Dinfo%253Apmid%252F24114695%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [6]: /lookup/external-ref?access_num=10.1007/s40272-013-0052-2&link_type=DOI [7]: /lookup/external-ref?access_num=24114695&link_type=MED&atom=%2Fpedsinreview%2F37%2F3%2F129.atom [8]: {openurl}?query=rft.jtitle%253DEuropean%2BRespiratory%2BJournal%26rft.stitle%253DEur%2BRespir%2BJ%26rft.aulast%253DBarst%26rft.auinit1%253DR.%2BJ.%26rft.volume%253D37%26rft.issue%253D3%26rft.spage%253D665%26rft.epage%253D677%26rft.atitle%253DPulmonary%2Barterial%2Bhypertension%253A%2Ba%2Bcomparison%2Bbetween%2Bchildren%2Band%2Badults%26rft_id%253Dinfo%253Adoi%252F10.1183%252F09031936.00056110%26rft_id%253Dinfo%253Apmid%252F21357924%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [9]: /lookup/ijlink?linkType=ABST&journalCode=erj&resid=37/3/665&atom=%2Fpedsinreview%2F37%2F3%2F129.atom