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type=\u0022text\/css\u0022 rel=\u0022stylesheet\u0022 href=\u0022\/\/d282kpwvnogo5m.cloudfront.net\/sites\/default\/files\/cdn\/css\/http\/css_Xg7z6oCTVgud_Q0huYz9x9iiD5H_2YPSJ5z2ZViSWdY.css\u0022 media=\u0022all\u0022 \/\u003E\n\u003Clink rel=\u0027stylesheet\u0027 type=\u0027text\/css\u0027 href=\u0027\/sites\/all\/modules\/contrib\/panels\/plugins\/layouts\/onecol\/onecol.css\u0027 \/\u003E\u003C\/head\u003E\u003Cbody\u003E\u003Cdiv class=\u0022panels-ajax-tab-panel panels-ajax-tab-panel-sageoa-tab-art\u0022\u003E\u003Cdiv class=\u0022panel-display panel-1col clearfix\u0022 \u003E\n  \u003Cdiv class=\u0022panel-panel panel-col\u0022\u003E\n    \u003Cdiv\u003E\u003Cdiv class=\u0022panel-pane pane-highwire-markup\u0022 \u003E\n  \n      \n  \n  \u003Cdiv class=\u0022pane-content\u0022\u003E\n    \u003Cdiv class=\u0022highwire-markup\u0022\u003E\u003Cdiv xmlns=\u0022http:\/\/www.w3.org\/1999\/xhtml\u0022 id=\u0022content-block-markup\u0022 xmlns:xhtml=\u0022http:\/\/www.w3.org\/1999\/xhtml\u0022\u003E\u003Cdiv class=\u0022article fulltext-view \u0022\u003E\u003Cspan class=\u0022highwire-journal-article-marker-start\u0022\u003E\u003C\/span\u003E\u003Cdiv class=\u0022section abstract\u0022 id=\u0022abstract-1\u0022\u003E\u003Ch2\u003ESummary\u003C\/h2\u003E\n            \u003Cp id=\u0022p-1\u0022\u003EGlobally, pulmonary vascular disease associated with congenital heart disease (CHD) may be the most preventable cause of pulmonary artery hypertension and related mortality and morbidity [Adatia I et al. \u003Cem\u003EChest\u003C\/em\u003E 2010]. This article presents an overview of advanced and combination therapies in patients with pulmonary arterial hypertension-CHD.\u003C\/p\u003E\n         \u003C\/div\u003E\u003Cul class=\u0022kwd-group\u0022\u003E\u003Cli class=\u0022kwd\u0022\u003EHypertensive Disease\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003ECardiology\u003C\/li\u003E\u003Cli class=\u0022kwd\u0022\u003EThromboembolic Disease\u003C\/li\u003E\u003C\/ul\u003E\u003Cp id=\u0022p-2\u0022\u003EGlobally, pulmonary vascular disease associated with congenital heart disease (CHD) may be the most preventable cause of pulmonary artery hypertension and related mortality and morbidity [Adatia I et al. \u003Cem\u003EChest\u003C\/em\u003E 2010]. Mohammed Omar Galal, MD, PhD, MBA, Prince Salman Heart Center, Riyadh, Saudi Arabia, presented an overview of advanced and combination therapies in patients with pulmonary arterial hypertension (PAH)-CHD.\u003C\/p\u003E\u003Cp id=\u0022p-3\u0022\u003EAlthough progress in the diagnosis and treatment of CHD has reduced the number of PAH-CHD cases in Western nations, few PAH patients in developing countries have access to treatment. Adatia et al. [\u003Cem\u003EChest\u003C\/em\u003E 2010] estimate that 3 million children worldwide are at risk for the development of pulmonary vascular disease due to CHD; most have a reparable heart defect, such as an isolated atrial septum, ventricular septal defect, or patent ductus arteriosus.\u003C\/p\u003E\u003Cp id=\u0022p-4\u0022\u003EPAH-CHD is classified into 4 types suitable for advanced therapy. These include 1) Eisenmenger Syndrome, 2) moderate to large shunt lesions with PAH, 3) small defects with PAH, and 4) PAH after repair of CHD [Galie N et al. \u003Cem\u003EEur Heart J\u003C\/em\u003E 2009; Simonneau G et al. \u003Cem\u003EJ Am Coll Cardiol\u003C\/em\u003E 2009]. Eisenmenger Syndrome is the most advanced form of pulmonary vascular disease secondary to CHD [Adatia I et al. \u003Cem\u003EChest\u003C\/em\u003E 2010].\u003C\/p\u003E\u003Cp id=\u0022p-5\u0022\u003ETreatment options in PAH range from general measures to advanced therapies (\u003Ca id=\u0022xref-fig-1-1\u0022 class=\u0022xref-fig\u0022 href=\u0022#F1\u0022\u003EFigure 1\u003C\/a\u003E). Conventional treatments include diuretics, anticoagulants, oxygen therapy, digoxin, and calcium channel blockers. In a randomized trial with 2 years of follow-up, Sandoval et al. [\u003Cem\u003EAm J Respir Crit Care Med\u003C\/em\u003E 2001] found that nocturnal oxygen therapy did not modify the natural history of patients with advanced Eisenmenger Syndrome. Calcium channel blockers benefit around 10% of patients with PAH and seem to improve survival, said Prof. Galal.\u003C\/p\u003E\u003Cdiv id=\u0022F1\u0022 class=\u0022fig pos-float  odd\u0022\u003E\u003Cdiv class=\u0022highwire-figure\u0022\u003E\u003Cdiv class=\u0022fig-inline-img-wrapper\u0022\u003E\u003Cdiv class=\u0022fig-inline-img\u0022\u003E\u003Ca href=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/12\/8\/27.1\/F1.large.jpg?width=800\u0026amp;height=600\u0026amp;carousel=1\u0022 title=\u0022Treatment Options in PAH*.\u0022 class=\u0022fragment-images colorbox-load\u0022 rel=\u0022gallery-fragment-images-743894217\u0022 data-figure-caption=\u0022Treatment Options in PAH*.\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003E\u003Cimg class=\u0022fragment-image\u0022 alt=\u0022Figure 1.\u0022 src=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/12\/8\/27.1\/F1.medium.gif\u0022\/\u003E\u003C\/a\u003E\u003C\/div\u003E\u003C\/div\u003E\u003Cul class=\u0022highwire-figure-links inline\u0022\u003E\u003Cli class=\u00220 first\u0022\u003E\u003Ca href=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/12\/8\/27.1\/F1.large.jpg?download=true\u0022 class=\u0022highwire-figure-link highwire-figure-link-download\u0022 title=\u0022Download Figure 1.\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EDownload figure\u003C\/a\u003E\u003C\/li\u003E\u003Cli class=\u00221\u0022\u003E\u003Ca href=\u0022http:\/\/d282kpwvnogo5m.cloudfront.net\/content\/spmdc\/12\/8\/27.1\/F1.large.jpg\u0022 class=\u0022highwire-figure-link highwire-figure-link-newtab\u0022 target=\u0022_blank\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EOpen in new tab\u003C\/a\u003E\u003C\/li\u003E\u003Cli class=\u00222 last\u0022\u003E\u003Ca href=\u0022\/highwire\/powerpoint\/12776\u0022 class=\u0022highwire-figure-link highwire-figure-link-ppt\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EDownload powerpoint\u003C\/a\u003E\u003C\/li\u003E\u003C\/ul\u003E\u003C\/div\u003E\u003Cdiv class=\u0022fig-caption attrib\u0022\u003E\u003Cspan class=\u0022fig-label\u0022\u003EFigure 1.\u003C\/span\u003E \n            \u003Cp id=\u0022p-6\u0022 class=\u0022first-child\u0022\u003ETreatment Options in PAH*.\u003C\/p\u003E\n         \u003Cq class=\u0022attrib\u0022 id=\u0022attrib-1\u0022\u003EPAH=pulmonary arterial hypertension; PDE-5=phosphodiesterase 5.\u003C\/q\u003E\u003Cdiv class=\u0022sb-div caption-clear\u0022\u003E\u003C\/div\u003E\u003C\/div\u003E\u003C\/div\u003E\u003Cp id=\u0022p-7\u0022\u003EAccording to Prof. Galal, advanced therapy should be used if there is a negative vasoreactivity test or lack of clinical improvement with calcium channel blockers. It can also be used in all groups with a NYHA functional class of II, III, or IV. However, most data on advanced care are based on studies with idiopathic PAH and PAH due to scleroderma, and the therapies are very expensive.\u003C\/p\u003E\u003Cp id=\u0022p-8\u0022\u003EThe 3 classes of advanced therapy include endothelin-1 receptor antagonists, phosphodiesterase 5 inhibitors, and prostanoids. Bosenten has been demonstrated to improve exercise capacity and stroke volume in patients without Down syndrome [Rubin LJ et al. \u003Cem\u003EN Engl J Med\u003C\/em\u003E 2002]. In a medium-term follow-up study of adult patients with PAH associated with CHD, Duffels et al. [\u003Cem\u003ECongenit Heart Dis\u003C\/em\u003E 2007] found that advanced treatment seemed to stabilize disease and decrease the rate of deterioration, especially in younger patients.\u003C\/p\u003E\u003Cp id=\u0022p-9\u0022\u003EBased on these and other data, Prof. Galal concluded that PAH-CHD remains a serious disease leading to reduced quality of life and longevity, 4 classes of PAH-CHD justify advanced therapies, that short-term studies have confirmed the efficacy and safety of advanced\/combination therapies, and long-term studies are still needed.\u003C\/p\u003E\u003Cul class=\u0022copyright-statement\u0022\u003E\u003Cli class=\u0022fn\u0022 id=\u0022copyright-statement-1\u0022\u003E\u00a9 2012 MD Conference Express\u00ae\u003C\/li\u003E\u003C\/ul\u003E\u003Cspan class=\u0022highwire-journal-article-marker-end\u0022\u003E\u003C\/span\u003E\u003C\/div\u003E\u003Cspan id=\u0022related-urls\u0022\u003E\u003C\/span\u003E\u003C\/div\u003E\u003Ca href=\u0022http:\/\/mdc.sagepub.com\/content\/12\/8\/27.1.abstract\u0022 class=\u0022hw-link hw-link-article-abstract\u0022 data-icon-position=\u0022\u0022 data-hide-link-title=\u00220\u0022\u003EView Summary\u003C\/a\u003E\u003C\/div\u003E  \u003C\/div\u003E\n\n  \n  \u003C\/div\u003E\n\u003C\/div\u003E\n  \u003C\/div\u003E\n\u003C\/div\u003E\n\u003C\/div\u003E\u003Cscript type=\u0022text\/javascript\u0022 src=\u0022http:\/\/mdc.sagepub.com\/sites\/all\/modules\/highwire\/highwire\/plugins\/highwire_markup_process\/js\/highwire_figures.js?nznakp\u0022\u003E\u003C\/script\u003E\n\u003Cscript type=\u0022text\/javascript\u0022 src=\u0022http:\/\/mdc.sagepub.com\/sites\/all\/modules\/highwire\/highwire\/plugins\/highwire_markup_process\/js\/highwire_openurl.js?nznakp\u0022\u003E\u003C\/script\u003E\n\u003C\/body\u003E\u003C\/html\u003E"}