Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre
BACKGROUND—Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of pat...
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| Veröffentlicht in: | Circulation (New York, N.Y.) N.Y.), 2015-12, Vol.132 (22), p.2118-2125 |
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| creator | Diller, Gerhard-Paul Kempny, Aleksander Alonso-Gonzalez, Rafael Swan, Lorna Uebing, Anselm Li, Wei Babu-Narayan, Sonya Wort, Stephen J Dimopoulos, Konstantinos Gatzoulis, Michael A |
| description | BACKGROUND—Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.
METHODS AND RESULTS—We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P |
| doi_str_mv | 10.1161/CIRCULATIONAHA.115.017202 |
| format | Article |
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METHODS AND RESULTS—We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient’s age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.
CONCLUSIONS—ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.</description><identifier>ISSN: 0009-7322</identifier><identifier>EISSN: 1524-4539</identifier><identifier>DOI: 10.1161/CIRCULATIONAHA.115.017202</identifier><identifier>PMID: 26369353</identifier><language>eng</language><publisher>United States: by the American College of Cardiology Foundation and the American Heart Association, Inc</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Cause of Death - trends ; Cohort Studies ; Female ; Follow-Up Studies ; Heart Defects, Congenital - diagnosis ; Heart Defects, Congenital - mortality ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Survival Rate - trends ; Tertiary Care Centers - trends ; Young Adult</subject><ispartof>Circulation (New York, N.Y.), 2015-12, Vol.132 (22), p.2118-2125</ispartof><rights>2015 by the American College of Cardiology Foundation and the American Heart Association, Inc.</rights><rights>2015 American Heart Association, Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4922-1f10ad65fa392664ecb1ef8bba58e317e583e71643291bf9630a1ab57c52c1593</citedby><cites>FETCH-LOGICAL-c4922-1f10ad65fa392664ecb1ef8bba58e317e583e71643291bf9630a1ab57c52c1593</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,3674,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26369353$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Diller, Gerhard-Paul</creatorcontrib><creatorcontrib>Kempny, Aleksander</creatorcontrib><creatorcontrib>Alonso-Gonzalez, Rafael</creatorcontrib><creatorcontrib>Swan, Lorna</creatorcontrib><creatorcontrib>Uebing, Anselm</creatorcontrib><creatorcontrib>Li, Wei</creatorcontrib><creatorcontrib>Babu-Narayan, Sonya</creatorcontrib><creatorcontrib>Wort, Stephen J</creatorcontrib><creatorcontrib>Dimopoulos, Konstantinos</creatorcontrib><creatorcontrib>Gatzoulis, Michael A</creatorcontrib><title>Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre</title><title>Circulation (New York, N.Y.)</title><addtitle>Circulation</addtitle><description>BACKGROUND—Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.
METHODS AND RESULTS—We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient’s age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.
CONCLUSIONS—ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Cause of Death - trends</subject><subject>Cohort Studies</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Heart Defects, Congenital - diagnosis</subject><subject>Heart Defects, Congenital - mortality</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Retrospective Studies</subject><subject>Survival Rate - trends</subject><subject>Tertiary Care Centers - trends</subject><subject>Young Adult</subject><issn>0009-7322</issn><issn>1524-4539</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkU1v1DAQhi0EokvhLyBz45LijziJDxyitGVXWtEKds_RJJl0Dc4HttMV_4KfjFdbkLhxGs2rZ94ZzUvIO86uOM_4h2rzpdpvy93m7nO5LqOmrhjPBRPPyIorkSapkvo5WTHGdJJLIS7IK--_xTaTuXpJLkQmMy2VXJFfXxf3aB7B0ns3-Rnb4CmMHa2Ma5fBBxhb9HTq6TVCOFAz0moaAw7z5MD9pGW32HCSHnA0IbqsEVyg18YjeKT3EAyO0XI_dujo7WTtdEz2M4VAgW7BPSDdoQvm5FVF0uFr8qIH6_HNU70k-9ubXbVOtnefNlW5TdpUC5HwnjPoMtWD1CLLUmwbjn3RNKAKlDxHVUjMeZZKoXnT60wy4NCovFWi5UrLS_L-7Du76ceCPtSD8S1aCyNOi695LotCsFTIiOoz2sYXeYd9PTszxJNrzupTIPW_gURN1edA4uzbpzVLM2D3d_JPAhH4eAaOkw3o_He7HNHVBwQbDv-x4DfEtp0q</recordid><startdate>20151201</startdate><enddate>20151201</enddate><creator>Diller, Gerhard-Paul</creator><creator>Kempny, Aleksander</creator><creator>Alonso-Gonzalez, Rafael</creator><creator>Swan, Lorna</creator><creator>Uebing, Anselm</creator><creator>Li, Wei</creator><creator>Babu-Narayan, Sonya</creator><creator>Wort, Stephen J</creator><creator>Dimopoulos, Konstantinos</creator><creator>Gatzoulis, Michael A</creator><general>by the American College of Cardiology Foundation and the American Heart Association, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20151201</creationdate><title>Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre</title><author>Diller, Gerhard-Paul ; Kempny, Aleksander ; Alonso-Gonzalez, Rafael ; Swan, Lorna ; Uebing, Anselm ; Li, Wei ; Babu-Narayan, Sonya ; Wort, Stephen J ; Dimopoulos, Konstantinos ; Gatzoulis, Michael A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4922-1f10ad65fa392664ecb1ef8bba58e317e583e71643291bf9630a1ab57c52c1593</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Cause of Death - trends</topic><topic>Cohort Studies</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Heart Defects, Congenital - diagnosis</topic><topic>Heart Defects, Congenital - mortality</topic><topic>Humans</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Retrospective Studies</topic><topic>Survival Rate - trends</topic><topic>Tertiary Care Centers - trends</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Diller, Gerhard-Paul</creatorcontrib><creatorcontrib>Kempny, Aleksander</creatorcontrib><creatorcontrib>Alonso-Gonzalez, Rafael</creatorcontrib><creatorcontrib>Swan, Lorna</creatorcontrib><creatorcontrib>Uebing, Anselm</creatorcontrib><creatorcontrib>Li, Wei</creatorcontrib><creatorcontrib>Babu-Narayan, Sonya</creatorcontrib><creatorcontrib>Wort, Stephen J</creatorcontrib><creatorcontrib>Dimopoulos, Konstantinos</creatorcontrib><creatorcontrib>Gatzoulis, Michael A</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Circulation (New York, N.Y.)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Diller, Gerhard-Paul</au><au>Kempny, Aleksander</au><au>Alonso-Gonzalez, Rafael</au><au>Swan, Lorna</au><au>Uebing, Anselm</au><au>Li, Wei</au><au>Babu-Narayan, Sonya</au><au>Wort, Stephen J</au><au>Dimopoulos, Konstantinos</au><au>Gatzoulis, Michael A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre</atitle><jtitle>Circulation (New York, N.Y.)</jtitle><addtitle>Circulation</addtitle><date>2015-12-01</date><risdate>2015</risdate><volume>132</volume><issue>22</issue><spage>2118</spage><epage>2125</epage><pages>2118-2125</pages><issn>0009-7322</issn><eissn>1524-4539</eissn><abstract>BACKGROUND—Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.
METHODS AND RESULTS—We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient’s age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.
CONCLUSIONS—ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.</abstract><cop>United States</cop><pub>by the American College of Cardiology Foundation and the American Heart Association, Inc</pub><pmid>26369353</pmid><doi>10.1161/CIRCULATIONAHA.115.017202</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Circulation (New York, N.Y.), 2015-12, Vol.132 (22), p.2118-2125 |
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| source | MEDLINE; American Heart Association Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Journals@Ovid Complete |
| subjects | Adolescent Adult Aged Aged, 80 and over Cause of Death - trends Cohort Studies Female Follow-Up Studies Heart Defects, Congenital - diagnosis Heart Defects, Congenital - mortality Humans Male Middle Aged Retrospective Studies Survival Rate - trends Tertiary Care Centers - trends Young Adult |
| title | Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre |
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