{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T19:16:37Z","timestamp":1777144597051,"version":"3.51.4"},"reference-count":30,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"17","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2024,9,3]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Despite a rigorous screening process, including cardiac catheterization, a subset of patients with a single right ventricle (SRV) demonstrates suboptimal short\u2010term outcomes after the Fontan operation. The goal of this study was to perform a comprehensive assessment of diastolic function in pre\u2010Fontan patients with an SRV using invasive reference\u2010standard measures and determine their associations with post\u2010Fontan outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      Children aged 2 to 6\u2009years with SRV physiology undergoing pre\u2010Fontan heart catheterization were recruited prospectively. Patients were divided into those who had an optimal or suboptimal outcome. A suboptimal outcome was defined as length of stay \u226514\u2009days or heart transplant\/cardiac death in first year after Fontan. Patients underwent pressure\u2013volume loop analysis using reference\u2010standard methods. The measure of ventricular stiffness, \u03b2, was obtained via preload reduction. Cardiac magnetic resonance imaging for extracellular volume and serum draws for matrix metalloproteinase activity were performed. Of 19 patients with an SRV, 9 (47%) had a suboptimal outcome. Mean age was 4.2\u00b10.7\u2009years. Patients with suboptimal outcomes had lower ventricular stiffness (0.021 [0.009\u20130.049] versus 0.090 [0.031\u20130.118] mL\n                      <jats:sup>\u22121<\/jats:sup>\n                      ;\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.02), lower extracellular volume (25% [28%\u201332%] versus 31% [28%\u201333%];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.02), and lower matrix metalloproteinase\u20102 (90 [79\u2013104] versus 108 [79\u2013128] ng\/mL;\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.01) compared with patients with optimal outcomes. The only invasive measure that had an association with suboptimal outcome was \u03b2 (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.038).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Patients with an SRV with suboptimal outcome after the Fontan operation had lower ventricular stiffness and evidence of maladaptive extracellular matrix metabolism compared with patients with optimal outcome. This appears to be a novel phenotype that may have important clinical implications and requires further study.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.124.035601","type":"journal-article","created":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T07:06:23Z","timestamp":1724742383000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Low Ventricular Stiffness Is Associated With Suboptimal Outcomes in Patients With a Single Right Ventricle After the Fontan Operation: A Novel Phenotype"],"prefix":"10.1161","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7685-7877","authenticated-orcid":false,"given":"Shahryar M.","family":"Chowdhury","sequence":"first","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4744-3832","authenticated-orcid":false,"given":"Andrew M.","family":"Atz","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric M.","family":"Graham","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Varsha M.","family":"Bandisode","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2928-6316","authenticated-orcid":false,"given":"John F.","family":"Rhodes","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arni C.","family":"Nutting","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolyn","family":"Taylor","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0422-1121","authenticated-orcid":false,"given":"Andrew","family":"Savage","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4491-8609","authenticated-orcid":false,"given":"Marc","family":"Hassid","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Minoo","family":"Kavarana","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donald","family":"Menick","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina","place":["Charleston, USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.78.4.376"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2003.06.018"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2012.01.003"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8175.2009.01085.x"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2004.04.008"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951109990382"},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2003.06.006"},{"key":"e_1_3_1_9_2","first-page":"IV164","article-title":"Fontan operation in 176 patients with tricuspid atresia. 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