Clinical and hemodynamic characteristics of the pediatric failing Fontan
To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant. In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were class...
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| Veröffentlicht in: | The Journal of heart and lung transplantation 2021-12, Vol.40 (12), p.1529-1539 |
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| container_title | The Journal of heart and lung transplantation |
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| creator | Dykes, John C. Rosenthal, David N. Bernstein, Daniel McElhinney, Doff B. Chrisant, Maryanne R.K. Daly, Kevin P. Ameduri, Rebecca K. Knecht, Kenneth Richmond, Marc E. Lin, Kimberly Y. Urschel, Simon Simmonds, Jacob Simpson, Kathleen E. Albers, Erin L. Khan, Asma Schumacher, Kurt Almond, Christopher S. Chen, Sharon |
| description | To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.
In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with “normal” hearts (FNH). Primary outcome was waitlist and post-transplant mortality.
177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI |
| doi_str_mv | 10.1016/j.healun.2021.07.017 |
| format | Article |
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In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with “normal” hearts (FNH). Primary outcome was waitlist and post-transplant mortality.
177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m2. FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%).
Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various phenotypes of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype.</description><identifier>ISSN: 1053-2498</identifier><identifier>EISSN: 1557-3117</identifier><identifier>DOI: 10.1016/j.healun.2021.07.017</identifier><identifier>PMID: 34412962</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adolescent ; Age Factors ; Child ; Child, Preschool ; Female ; Fontan ; Fontan Procedure - adverse effects ; Heart Defects, Congenital - mortality ; Heart Defects, Congenital - physiopathology ; Heart Defects, Congenital - surgery ; heart failure ; heart transplant ; Heart Transplantation ; Hemodynamics ; Humans ; Male ; Retrospective Studies ; Risk Factors ; single ventricle ; Survival Rate ; Treatment Failure ; Waiting Lists</subject><ispartof>The Journal of heart and lung transplantation, 2021-12, Vol.40 (12), p.1529-1539</ispartof><rights>2021 International Society for Heart and Lung Transplantation</rights><rights>Copyright © 2021 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c362t-7770df2b4c67ce4a8128f054397ea80c9a3ba3cde9157b926a6f0e856accb0353</citedby><cites>FETCH-LOGICAL-c362t-7770df2b4c67ce4a8128f054397ea80c9a3ba3cde9157b926a6f0e856accb0353</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.healun.2021.07.017$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34412962$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Dykes, John C.</creatorcontrib><creatorcontrib>Rosenthal, David N.</creatorcontrib><creatorcontrib>Bernstein, Daniel</creatorcontrib><creatorcontrib>McElhinney, Doff B.</creatorcontrib><creatorcontrib>Chrisant, Maryanne R.K.</creatorcontrib><creatorcontrib>Daly, Kevin P.</creatorcontrib><creatorcontrib>Ameduri, Rebecca K.</creatorcontrib><creatorcontrib>Knecht, Kenneth</creatorcontrib><creatorcontrib>Richmond, Marc E.</creatorcontrib><creatorcontrib>Lin, Kimberly Y.</creatorcontrib><creatorcontrib>Urschel, Simon</creatorcontrib><creatorcontrib>Simmonds, Jacob</creatorcontrib><creatorcontrib>Simpson, Kathleen E.</creatorcontrib><creatorcontrib>Albers, Erin L.</creatorcontrib><creatorcontrib>Khan, Asma</creatorcontrib><creatorcontrib>Schumacher, Kurt</creatorcontrib><creatorcontrib>Almond, Christopher S.</creatorcontrib><creatorcontrib>Chen, Sharon</creatorcontrib><creatorcontrib>Pediatric Heart Transplant Society</creatorcontrib><title>Clinical and hemodynamic characteristics of the pediatric failing Fontan</title><title>The Journal of heart and lung transplantation</title><addtitle>J Heart Lung Transplant</addtitle><description>To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.
In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with “normal” hearts (FNH). Primary outcome was waitlist and post-transplant mortality.
177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m2. FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%).
Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various phenotypes of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype.</description><subject>Adolescent</subject><subject>Age Factors</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Female</subject><subject>Fontan</subject><subject>Fontan Procedure - adverse effects</subject><subject>Heart Defects, Congenital - mortality</subject><subject>Heart Defects, Congenital - physiopathology</subject><subject>Heart Defects, Congenital - surgery</subject><subject>heart failure</subject><subject>heart transplant</subject><subject>Heart Transplantation</subject><subject>Hemodynamics</subject><subject>Humans</subject><subject>Male</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>single ventricle</subject><subject>Survival Rate</subject><subject>Treatment Failure</subject><subject>Waiting Lists</subject><issn>1053-2498</issn><issn>1557-3117</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE1LxDAQhoMofv8DkR69tOajSdqLIIurguBFz2E6nbpZ-rEmXcF_b2TVo6eZw_POyzyMXQheCC7M9bpYEfTbsZBcioLbggu7x46F1jZXQtj9tHOtclnW1RE7iXHNOZdKy0N2pMpSyNrIY_aw6P3oEfoMxjZb0TC1nyMMHjNcQQCcKfg4e4zZ1GXzirINtR7mkIAOfMq-ZctpnGE8Ywcd9JHOf-Ype13evSwe8qfn-8fF7VOOysg5t9bytpNNicYilVAJWXVcl6q2BBXHGlQDCluqhbZNLQ2YjlOlDSA2XGl1yq52dzdhet9SnN3gI1Lfw0jTNjqpjSqlNcYktNyhGKYYA3VuE_wA4dMJ7r4durXbOXTfDh23LjlMscufhm0zUPsX-pWWgJsdQOnPD0_BRfQ0YjITCGfXTv7_hi-YJIR8</recordid><startdate>202112</startdate><enddate>202112</enddate><creator>Dykes, John C.</creator><creator>Rosenthal, David N.</creator><creator>Bernstein, Daniel</creator><creator>McElhinney, Doff B.</creator><creator>Chrisant, Maryanne R.K.</creator><creator>Daly, Kevin P.</creator><creator>Ameduri, Rebecca K.</creator><creator>Knecht, Kenneth</creator><creator>Richmond, Marc E.</creator><creator>Lin, Kimberly Y.</creator><creator>Urschel, Simon</creator><creator>Simmonds, Jacob</creator><creator>Simpson, Kathleen E.</creator><creator>Albers, Erin L.</creator><creator>Khan, Asma</creator><creator>Schumacher, Kurt</creator><creator>Almond, Christopher S.</creator><creator>Chen, Sharon</creator><general>Elsevier 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>202112</creationdate><title>Clinical and hemodynamic characteristics of the pediatric failing Fontan</title><author>Dykes, John C. ; Rosenthal, David N. ; Bernstein, Daniel ; McElhinney, Doff B. ; Chrisant, Maryanne R.K. ; Daly, Kevin P. ; Ameduri, Rebecca K. ; Knecht, Kenneth ; Richmond, Marc E. ; Lin, Kimberly Y. ; Urschel, Simon ; Simmonds, Jacob ; Simpson, Kathleen E. ; Albers, Erin L. ; Khan, Asma ; Schumacher, Kurt ; Almond, Christopher S. ; Chen, Sharon</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c362t-7770df2b4c67ce4a8128f054397ea80c9a3ba3cde9157b926a6f0e856accb0353</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Adolescent</topic><topic>Age Factors</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Female</topic><topic>Fontan</topic><topic>Fontan Procedure - adverse effects</topic><topic>Heart Defects, Congenital - mortality</topic><topic>Heart Defects, Congenital - physiopathology</topic><topic>Heart Defects, Congenital - surgery</topic><topic>heart failure</topic><topic>heart transplant</topic><topic>Heart Transplantation</topic><topic>Hemodynamics</topic><topic>Humans</topic><topic>Male</topic><topic>Retrospective Studies</topic><topic>Risk Factors</topic><topic>single ventricle</topic><topic>Survival Rate</topic><topic>Treatment Failure</topic><topic>Waiting Lists</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Dykes, John C.</creatorcontrib><creatorcontrib>Rosenthal, David N.</creatorcontrib><creatorcontrib>Bernstein, Daniel</creatorcontrib><creatorcontrib>McElhinney, Doff B.</creatorcontrib><creatorcontrib>Chrisant, Maryanne R.K.</creatorcontrib><creatorcontrib>Daly, Kevin P.</creatorcontrib><creatorcontrib>Ameduri, Rebecca K.</creatorcontrib><creatorcontrib>Knecht, Kenneth</creatorcontrib><creatorcontrib>Richmond, Marc E.</creatorcontrib><creatorcontrib>Lin, Kimberly Y.</creatorcontrib><creatorcontrib>Urschel, Simon</creatorcontrib><creatorcontrib>Simmonds, Jacob</creatorcontrib><creatorcontrib>Simpson, Kathleen E.</creatorcontrib><creatorcontrib>Albers, Erin L.</creatorcontrib><creatorcontrib>Khan, Asma</creatorcontrib><creatorcontrib>Schumacher, Kurt</creatorcontrib><creatorcontrib>Almond, Christopher S.</creatorcontrib><creatorcontrib>Chen, Sharon</creatorcontrib><creatorcontrib>Pediatric Heart Transplant Society</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>The Journal of heart and lung transplantation</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Dykes, John C.</au><au>Rosenthal, David N.</au><au>Bernstein, Daniel</au><au>McElhinney, Doff B.</au><au>Chrisant, Maryanne R.K.</au><au>Daly, Kevin P.</au><au>Ameduri, Rebecca K.</au><au>Knecht, Kenneth</au><au>Richmond, Marc E.</au><au>Lin, Kimberly Y.</au><au>Urschel, Simon</au><au>Simmonds, Jacob</au><au>Simpson, Kathleen E.</au><au>Albers, Erin L.</au><au>Khan, Asma</au><au>Schumacher, Kurt</au><au>Almond, Christopher S.</au><au>Chen, Sharon</au><aucorp>Pediatric Heart Transplant Society</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical and hemodynamic characteristics of the pediatric failing Fontan</atitle><jtitle>The Journal of heart and lung transplantation</jtitle><addtitle>J Heart Lung Transplant</addtitle><date>2021-12</date><risdate>2021</risdate><volume>40</volume><issue>12</issue><spage>1529</spage><epage>1539</epage><pages>1529-1539</pages><issn>1053-2498</issn><eissn>1557-3117</eissn><abstract>To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.
In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with “normal” hearts (FNH). Primary outcome was waitlist and post-transplant mortality.
177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m2. FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%).
Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various phenotypes of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>34412962</pmid><doi>10.1016/j.healun.2021.07.017</doi><tpages>11</tpages></addata></record> |
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| subjects | Adolescent Age Factors Child Child, Preschool Female Fontan Fontan Procedure - adverse effects Heart Defects, Congenital - mortality Heart Defects, Congenital - physiopathology Heart Defects, Congenital - surgery heart failure heart transplant Heart Transplantation Hemodynamics Humans Male Retrospective Studies Risk Factors single ventricle Survival Rate Treatment Failure Waiting Lists |
| title | Clinical and hemodynamic characteristics of the pediatric failing Fontan |
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