Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation
Abstract Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation. Methods From 1992 to 2010, 42 patie...
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| Veröffentlicht in: | Journal of the American College of Cardiology 2014-07, Vol.64 (1), p.54-62 |
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| description | Abstract Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation. Methods From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively. Results Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction 2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2 ; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]). Conclusions PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy. |
| doi_str_mv | 10.1016/j.jacc.2014.04.025 |
| format | Article |
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Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation. Methods From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively. Results Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2 ; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]). Conclusions PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.</description><identifier>ISSN: 0735-1097</identifier><identifier>EISSN: 1558-3597</identifier><identifier>DOI: 10.1016/j.jacc.2014.04.025</identifier><identifier>PMID: 24998129</identifier><identifier>CODEN: JACCDI</identifier><language>eng</language><publisher>New York, NY: Elsevier</publisher><subject>Adolescent ; Adult ; Age ; Biological and medical sciences ; Cardiology ; Cardiology. Vascular system ; Cardiovascular ; Child ; Child, Preschool ; Clinical outcomes ; Cohort Studies ; Female ; Fontan Procedure - mortality ; Fontan Procedure - trends ; Gastroenterology. Liver. Pancreas. Abdomen ; Heart ; Humans ; Infant ; Internal Medicine ; Intubation ; Male ; Medical sciences ; Middle Aged ; Mortality ; NMR ; Nuclear magnetic resonance ; Other diseases. Semiology ; Protein-Losing Enteropathies - mortality ; Protein-Losing Enteropathies - surgery ; Proteins ; Retrospective Studies ; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus ; Surgery ; Survival Rate - trends ; Treatment Outcome ; Young Adult</subject><ispartof>Journal of the American College of Cardiology, 2014-07, Vol.64 (1), p.54-62</ispartof><rights>American College of Cardiology Foundation</rights><rights>2015 INIST-CNRS</rights><rights>Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.</rights><rights>Copyright Elsevier Limited Jul 8, 2014</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c460t-4ee057e933775922b0f50519ef17b71f1fd3911c5d256fdfa61df5061021a7c13</citedby><cites>FETCH-LOGICAL-c460t-4ee057e933775922b0f50519ef17b71f1fd3911c5d256fdfa61df5061021a7c13</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=28600170$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24998129$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>John, Anitha S., MD, PhD</creatorcontrib><creatorcontrib>Johnson, Jennifer A., MD</creatorcontrib><creatorcontrib>Khan, Munziba, MPH</creatorcontrib><creatorcontrib>Driscoll, David J., MD</creatorcontrib><creatorcontrib>Warnes, Carole A., MD</creatorcontrib><creatorcontrib>Cetta, Frank, MD</creatorcontrib><title>Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation</title><title>Journal of the American College of Cardiology</title><addtitle>J Am Coll Cardiol</addtitle><description>Abstract Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation. Methods From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively. Results Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2 ; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]). Conclusions PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Age</subject><subject>Biological and medical sciences</subject><subject>Cardiology</subject><subject>Cardiology. Vascular system</subject><subject>Cardiovascular</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Clinical outcomes</subject><subject>Cohort Studies</subject><subject>Female</subject><subject>Fontan Procedure - mortality</subject><subject>Fontan Procedure - trends</subject><subject>Gastroenterology. Liver. Pancreas. Abdomen</subject><subject>Heart</subject><subject>Humans</subject><subject>Infant</subject><subject>Internal Medicine</subject><subject>Intubation</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Mortality</subject><subject>NMR</subject><subject>Nuclear magnetic resonance</subject><subject>Other diseases. Semiology</subject><subject>Protein-Losing Enteropathies - mortality</subject><subject>Protein-Losing Enteropathies - surgery</subject><subject>Proteins</subject><subject>Retrospective Studies</subject><subject>Stomach. Duodenum. Small intestine. Colon. Rectum. Anus</subject><subject>Surgery</subject><subject>Survival Rate - trends</subject><subject>Treatment Outcome</subject><subject>Young Adult</subject><issn>0735-1097</issn><issn>1558-3597</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkVGLEzEQxxdRvHr6BXyQgAi-bJ1JNrubF-Eod3pQ6MEpPoY0O2tT22xNsoV-e7O0eiAMJJDff5jMryjeIswRsP60nW-NtXMOWM0hF5fPihlK2ZZCquZ5MYNGyBJBNVfFqxi3AFC3qF4WV7xSqkWuZoVf7Jx31uzYakx22FNkxnfsfn8Iw5E69jiGozvmZ-fZg0mOfIrsh0sb9hCGRM6XyyE6_5Pd-kRhOJi0ObGbPt9Z2hC7G3wynq0OFHJ48K-LF73ZRXpzOa-L73e33xZfy-Xqy_3iZlnaqoZUVkQgG1JCNI1UnK-hlyBRUY_NusEe-04oRCs7Luu-602NXSZqBI6msSiui4_nvvkbv0eKSe9dtLTbGU_DGDXKStSt4C3P6Pv_0O0wBp-nmyheVa1AlSl-pmwYYgzU60NwexNOGkFPNvRWTzb0ZENDLi5z6N2l9bjeU_cv8nf9GfhwAUzMDvpgvHXxiWtrAGwgc5_PHOWdHR0FbS_aftGJ4tPAOnIN-nESP3nHCrgQFYo_MTinxw</recordid><startdate>20140708</startdate><enddate>20140708</enddate><creator>John, Anitha S., MD, PhD</creator><creator>Johnson, Jennifer A., MD</creator><creator>Khan, Munziba, MPH</creator><creator>Driscoll, David J., MD</creator><creator>Warnes, Carole A., MD</creator><creator>Cetta, Frank, MD</creator><general>Elsevier</general><general>Elsevier Limited</general><scope>IQODW</scope><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>7T5</scope><scope>7TK</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope></search><sort><creationdate>20140708</creationdate><title>Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation</title><author>John, Anitha S., MD, PhD ; Johnson, Jennifer A., MD ; Khan, Munziba, MPH ; Driscoll, David J., MD ; Warnes, Carole A., MD ; Cetta, Frank, MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c460t-4ee057e933775922b0f50519ef17b71f1fd3911c5d256fdfa61df5061021a7c13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Age</topic><topic>Biological and medical sciences</topic><topic>Cardiology</topic><topic>Cardiology. Vascular system</topic><topic>Cardiovascular</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Clinical outcomes</topic><topic>Cohort Studies</topic><topic>Female</topic><topic>Fontan Procedure - mortality</topic><topic>Fontan Procedure - trends</topic><topic>Gastroenterology. Liver. Pancreas. Abdomen</topic><topic>Heart</topic><topic>Humans</topic><topic>Infant</topic><topic>Internal Medicine</topic><topic>Intubation</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Mortality</topic><topic>NMR</topic><topic>Nuclear magnetic resonance</topic><topic>Other diseases. Semiology</topic><topic>Protein-Losing Enteropathies - mortality</topic><topic>Protein-Losing Enteropathies - surgery</topic><topic>Proteins</topic><topic>Retrospective Studies</topic><topic>Stomach. Duodenum. Small intestine. Colon. Rectum. Anus</topic><topic>Surgery</topic><topic>Survival Rate - trends</topic><topic>Treatment Outcome</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>John, Anitha S., MD, PhD</creatorcontrib><creatorcontrib>Johnson, Jennifer A., MD</creatorcontrib><creatorcontrib>Khan, Munziba, MPH</creatorcontrib><creatorcontrib>Driscoll, David J., MD</creatorcontrib><creatorcontrib>Warnes, Carole A., MD</creatorcontrib><creatorcontrib>Cetta, Frank, MD</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the American College of Cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>John, Anitha S., MD, PhD</au><au>Johnson, Jennifer A., MD</au><au>Khan, Munziba, MPH</au><au>Driscoll, David J., MD</au><au>Warnes, Carole A., MD</au><au>Cetta, Frank, MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation</atitle><jtitle>Journal of the American College of Cardiology</jtitle><addtitle>J Am Coll Cardiol</addtitle><date>2014-07-08</date><risdate>2014</risdate><volume>64</volume><issue>1</issue><spage>54</spage><epage>62</epage><pages>54-62</pages><issn>0735-1097</issn><eissn>1558-3597</eissn><coden>JACCDI</coden><abstract>Abstract Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation. Methods From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively. Results Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2 ; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]). Conclusions PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.</abstract><cop>New York, NY</cop><pub>Elsevier</pub><pmid>24998129</pmid><doi>10.1016/j.jacc.2014.04.025</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adolescent Adult Age Biological and medical sciences Cardiology Cardiology. Vascular system Cardiovascular Child Child, Preschool Clinical outcomes Cohort Studies Female Fontan Procedure - mortality Fontan Procedure - trends Gastroenterology. Liver. Pancreas. Abdomen Heart Humans Infant Internal Medicine Intubation Male Medical sciences Middle Aged Mortality NMR Nuclear magnetic resonance Other diseases. Semiology Protein-Losing Enteropathies - mortality Protein-Losing Enteropathies - surgery Proteins Retrospective Studies Stomach. Duodenum. Small intestine. Colon. Rectum. Anus Surgery Survival Rate - trends Treatment Outcome Young Adult |
| title | Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation |
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