Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts
To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. All prev...
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| Veröffentlicht in: | PloS one 2018-04, Vol.13 (4), p.e0195092-e0195092 |
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| creator | Vijarnsorn, Chodchanok Durongpisitkul, Kritvikrom Chungsomprasong, Paweena Bositthipichet, Densiri Ketsara, Salisa Titaram, Yuttapon Chanthong, Prakul Kanjanauthai, Supaluck Soongswang, Jarupim |
| description | To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort.
All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis.
A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2.
Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients. |
| doi_str_mv | 10.1371/journal.pone.0195092 |
| format | Article |
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All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis.
A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2.
Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0195092</identifier><identifier>PMID: 29664959</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Age ; Biology and Life Sciences ; Blood pressure ; Bypasses ; Cardiovascular disease ; Catheterization ; Congenital diseases ; Coronary artery disease ; Defects ; Epidemiology ; Heart ; Heart diseases ; Heart failure ; Hospitals ; Hypertension ; Intubation ; Medical prognosis ; Medicine ; Medicine and Health Sciences ; Mortality ; Multivariate analysis ; Patients ; Pediatrics ; Pulmonary arteries ; Pulmonary hypertension ; Shunts ; Statistical analysis ; Surgery ; Survival ; Ventricle</subject><ispartof>PloS one, 2018-04, Vol.13 (4), p.e0195092-e0195092</ispartof><rights>2018 Vijarnsorn et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2018 Vijarnsorn et al 2018 Vijarnsorn et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c526t-4d9da3df636070d250eb9849411f4928b28b4fe4cd6a830249cf8c6bf7409e103</citedby><cites>FETCH-LOGICAL-c526t-4d9da3df636070d250eb9849411f4928b28b4fe4cd6a830249cf8c6bf7409e103</cites><orcidid>0000-0001-7124-2064</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903600/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903600/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,724,777,781,861,882,2096,2915,23847,27905,27906,53772,53774,79349,79350</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29664959$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Fukumoto, Yoshihiro</contributor><creatorcontrib>Vijarnsorn, Chodchanok</creatorcontrib><creatorcontrib>Durongpisitkul, Kritvikrom</creatorcontrib><creatorcontrib>Chungsomprasong, Paweena</creatorcontrib><creatorcontrib>Bositthipichet, Densiri</creatorcontrib><creatorcontrib>Ketsara, Salisa</creatorcontrib><creatorcontrib>Titaram, Yuttapon</creatorcontrib><creatorcontrib>Chanthong, Prakul</creatorcontrib><creatorcontrib>Kanjanauthai, Supaluck</creatorcontrib><creatorcontrib>Soongswang, Jarupim</creatorcontrib><title>Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort.
All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis.
A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2.
Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.</description><subject>Age</subject><subject>Biology and Life Sciences</subject><subject>Blood pressure</subject><subject>Bypasses</subject><subject>Cardiovascular disease</subject><subject>Catheterization</subject><subject>Congenital diseases</subject><subject>Coronary artery disease</subject><subject>Defects</subject><subject>Epidemiology</subject><subject>Heart</subject><subject>Heart diseases</subject><subject>Heart failure</subject><subject>Hospitals</subject><subject>Hypertension</subject><subject>Intubation</subject><subject>Medical prognosis</subject><subject>Medicine</subject><subject>Medicine and Health Sciences</subject><subject>Mortality</subject><subject>Multivariate analysis</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Pulmonary arteries</subject><subject>Pulmonary hypertension</subject><subject>Shunts</subject><subject>Statistical analysis</subject><subject>Surgery</subject><subject>Survival</subject><subject>Ventricle</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>DOA</sourceid><recordid>eNptUl1r2zAUNWWl7br9g7EZ9tKXZPqy7PsyGGHdCoW9bM9C1keiYEueZKfk309p3JKOgkBC95xzz7ncoviA0RLTGn_Zhil62S2H4M0SYagQkLPiCgMlC04QfXPyvizeprRFqKIN5xfFJQHOGVRwVcRV8KPphxBl3Jdpiju3k10ZbDnI0Rk_pvLBjZtymLo--ANGxtFElzGb_WDy2ycXfCm9LlXwa-PdmGtpn7KqU-UYTqhpM2XBd8W5lV0y7-f7uvhz-_336ufi_tePu9W3-4WqCB8XTIOWVFtOOaqRJhUyLTQMGMaWAWnafJg1TGkuG4oIA2UbxVtbMwQGI3pdfDrqDl1IYh5XEgQRTgEBIhlxd0ToILdiiK7PLkWQTjx-hLgWOa1TnREVo5ozzcBqy6oGgGNrW5WbNw22UGetr3O3qe2NVnl0UXYvRF9WvNuIddiJClBOeLB7MwvE8HcyaRS9S8p0nfQmTI--a8QrAMjQz_9BX0_HjigVQ0rR2GczGInDCj2xxGGFxLxCmfbxNMgz6Wln6D_Klsgo</recordid><startdate>20180417</startdate><enddate>20180417</enddate><creator>Vijarnsorn, 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survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts</title><author>Vijarnsorn, Chodchanok ; Durongpisitkul, Kritvikrom ; Chungsomprasong, Paweena ; Bositthipichet, Densiri ; Ketsara, Salisa ; Titaram, Yuttapon ; Chanthong, Prakul ; Kanjanauthai, Supaluck ; Soongswang, Jarupim</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c526t-4d9da3df636070d250eb9849411f4928b28b4fe4cd6a830249cf8c6bf7409e103</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Age</topic><topic>Biology and Life Sciences</topic><topic>Blood pressure</topic><topic>Bypasses</topic><topic>Cardiovascular disease</topic><topic>Catheterization</topic><topic>Congenital diseases</topic><topic>Coronary artery disease</topic><topic>Defects</topic><topic>Epidemiology</topic><topic>Heart</topic><topic>Heart diseases</topic><topic>Heart failure</topic><topic>Hospitals</topic><topic>Hypertension</topic><topic>Intubation</topic><topic>Medical prognosis</topic><topic>Medicine</topic><topic>Medicine and Health Sciences</topic><topic>Mortality</topic><topic>Multivariate analysis</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Pulmonary arteries</topic><topic>Pulmonary hypertension</topic><topic>Shunts</topic><topic>Statistical analysis</topic><topic>Surgery</topic><topic>Survival</topic><topic>Ventricle</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vijarnsorn, Chodchanok</creatorcontrib><creatorcontrib>Durongpisitkul, Kritvikrom</creatorcontrib><creatorcontrib>Chungsomprasong, Paweena</creatorcontrib><creatorcontrib>Bositthipichet, Densiri</creatorcontrib><creatorcontrib>Ketsara, Salisa</creatorcontrib><creatorcontrib>Titaram, Yuttapon</creatorcontrib><creatorcontrib>Chanthong, Prakul</creatorcontrib><creatorcontrib>Kanjanauthai, 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Yoshihiro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2018-04-17</date><risdate>2018</risdate><volume>13</volume><issue>4</issue><spage>e0195092</spage><epage>e0195092</epage><pages>e0195092-e0195092</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort.
All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis.
A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2.
Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>29664959</pmid><doi>10.1371/journal.pone.0195092</doi><orcidid>https://orcid.org/0000-0001-7124-2064</orcidid><oa>free_for_read</oa></addata></record> |
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| issn | 1932-6203 1932-6203 |
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| source | PLoS; Full-Text Journals in Chemistry (Open access); DOAJ Directory of Open Access Journals; PubMed Central; EZB Electronic Journals Library |
| subjects | Age Biology and Life Sciences Blood pressure Bypasses Cardiovascular disease Catheterization Congenital diseases Coronary artery disease Defects Epidemiology Heart Heart diseases Heart failure Hospitals Hypertension Intubation Medical prognosis Medicine Medicine and Health Sciences Mortality Multivariate analysis Patients Pediatrics Pulmonary arteries Pulmonary hypertension Shunts Statistical analysis Surgery Survival Ventricle |
| title | Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-17T17%3A59%3A47IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Contemporary%20survival%20of%20patients%20with%20pulmonary%20arterial%20hypertension%20and%20congenital%20systemic%20to%20pulmonary%20shunts&rft.jtitle=PloS%20one&rft.au=Vijarnsorn,%20Chodchanok&rft.date=2018-04-17&rft.volume=13&rft.issue=4&rft.spage=e0195092&rft.epage=e0195092&rft.pages=e0195092-e0195092&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0195092&rft_dat=%3Cproquest_plos_%3E2027065999%3C/proquest_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2026390902&rft_id=info:pmid/29664959&rft_doaj_id=oai_doaj_org_article_543d64d49fdf4589961ffbccd6881f97&rfr_iscdi=true |