Central Venous Waveform Patterns in the Fontan Circulation Independently Contribute to the Prediction of Composite Survival

It is well appreciated that the Fontan circulation perturbs central venous hemodynamics, with elevated pressure being the clearest change associated with Fontan comorbidities, such as Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE). Our group has better quantity of these...

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Veröffentlicht in:Pediatric cardiology 2024-12, Vol.45 (8), p.1617-1626
Hauptverfasser: Ferrari, Margaret R., Schäfer, Michal, Hunter, Kendall S., Di Maria, Michael V.
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container_end_page 1626
container_issue 8
container_start_page 1617
container_title Pediatric cardiology
container_volume 45
creator Ferrari, Margaret R.
Schäfer, Michal
Hunter, Kendall S.
Di Maria, Michael V.
description It is well appreciated that the Fontan circulation perturbs central venous hemodynamics, with elevated pressure being the clearest change associated with Fontan comorbidities, such as Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE). Our group has better quantity of these venous perturbations through single- and multi-location analyses of flow waveforms obtained from magnetic resonance imaging of Fontan patients. Here, we determine if such analyses, which yield principal components (PC) that describe flow features, are associated with Fontan survival. Patients with a Fontan circulation ( N  = 140) that underwent free-breathing and mechanically ventilated cardiac MRI were included in this study. Standard volumetric and functional hemodynamics, as well as flow analysis principal components, were subjected to univariate and bivariate Cox regression analyses to determine composite clinical outcome, including plastic bronchitis, PLE, and referral and receipt of transplant. Unsurprisingly, ventricular function measures of ejection fraction (EF; HR = 0.88, p  
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Schäfer, Michal ; Hunter, Kendall S. ; Di Maria, Michael V.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c354t-bc8a360d4f1806defc485e6b0cb7b9ba9550f68a1f74b8d40be97f5b609a02023</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Adolescent</topic><topic>Cardiac Surgery</topic><topic>Cardiology</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Female</topic><topic>Fontan Procedure</topic><topic>Heart Defects, Congenital - physiopathology</topic><topic>Heart Defects, Congenital - surgery</topic><topic>Hemodynamics</topic><topic>Humans</topic><topic>Magnetic Resonance Imaging</topic><topic>Magnetic Resonance Imaging, Cine - methods</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine &amp; Public Health</topic><topic>Protein-Losing Enteropathies - etiology</topic><topic>Protein-Losing Enteropathies - physiopathology</topic><topic>Retrospective Studies</topic><topic>Review</topic><topic>Stroke Volume - physiology</topic><topic>Vascular Surgery</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ferrari, Margaret R.</creatorcontrib><creatorcontrib>Schäfer, Michal</creatorcontrib><creatorcontrib>Hunter, Kendall S.</creatorcontrib><creatorcontrib>Di Maria, Michael V.</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><collection>PubMed Central (Full Participant titles)</collection><jtitle>Pediatric cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ferrari, Margaret R.</au><au>Schäfer, Michal</au><au>Hunter, Kendall S.</au><au>Di Maria, Michael V.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Central Venous Waveform Patterns in the Fontan Circulation Independently Contribute to the Prediction of Composite Survival</atitle><jtitle>Pediatric cardiology</jtitle><stitle>Pediatr Cardiol</stitle><addtitle>Pediatr Cardiol</addtitle><date>2024-12-01</date><risdate>2024</risdate><volume>45</volume><issue>8</issue><spage>1617</spage><epage>1626</epage><pages>1617-1626</pages><issn>0172-0643</issn><issn>1432-1971</issn><eissn>1432-1971</eissn><abstract>It is well appreciated that the Fontan circulation perturbs central venous hemodynamics, with elevated pressure being the clearest change associated with Fontan comorbidities, such as Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE). Our group has better quantity of these venous perturbations through single- and multi-location analyses of flow waveforms obtained from magnetic resonance imaging of Fontan patients. Here, we determine if such analyses, which yield principal components (PC) that describe flow features, are associated with Fontan survival. Patients with a Fontan circulation ( N  = 140) that underwent free-breathing and mechanically ventilated cardiac MRI were included in this study. Standard volumetric and functional hemodynamics, as well as flow analysis principal components, were subjected to univariate and bivariate Cox regression analyses to determine composite clinical outcome, including plastic bronchitis, PLE, and referral and receipt of transplant. Unsurprisingly, ventricular function measures of ejection fraction (EF; HR = 0.88, p  &lt; 0.0001), indexed end-systolic volume (ESVi; HR 1.02, p  &lt; 0.0001), and indexed end-diastolic volume (EDVi; HR = 1.02, p  = 0.0007) were found as specific predictors of clinical events, with specificities uniformly &gt; 0.75. Additionally a feature of IVC flow (PC2) indicating increased flow in systole was found as a highly sensitive predictor (HR = 0.851, p  = 0.027, sensitivity 0.93). In bivariate prediction, combinations of ventricular function (EF, ESVi, EDVi) with this IVC flow feature yielded best overall prediction of composite outcome. This suggests that central venous waveform analysis relays additional information about Fontan patient survival and that coupling sensitive and specific measures in bivariate analysis is a useful approach for obtaining superior prediction of survival.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>37773462</pmid><doi>10.1007/s00246-023-03268-w</doi><tpages>10</tpages></addata></record>
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subjects Adolescent
Cardiac Surgery
Cardiology
Child
Child, Preschool
Female
Fontan Procedure
Heart Defects, Congenital - physiopathology
Heart Defects, Congenital - surgery
Hemodynamics
Humans
Magnetic Resonance Imaging
Magnetic Resonance Imaging, Cine - methods
Male
Medicine
Medicine & Public Health
Protein-Losing Enteropathies - etiology
Protein-Losing Enteropathies - physiopathology
Retrospective Studies
Review
Stroke Volume - physiology
Vascular Surgery
title Central Venous Waveform Patterns in the Fontan Circulation Independently Contribute to the Prediction of Composite Survival
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