{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T11:10:12Z","timestamp":1784113812984,"version":"3.55.0"},"reference-count":30,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2025,5,6]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      The heterozygous ring finger protein 213\n                      <jats:italic toggle=\"no\">(RNF213)<\/jats:italic>\n                      p.Arg4810Lys variant has been identified in patients with chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to clarify the influence of the\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant on the response to balloon pulmonary angioplasty (BPA) in patients with CTEPH.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      We retrospectively analyzed 93 patients with CTEPH who underwent BPA by analyzing the\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant. Clinical parameters and hemodynamics following BPA were compared between\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant carriers and noncarriers, along with BPA\u2010related complications.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      Among 93 patients, 7 (8%) were heterozygous\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant carriers and 86 (92%) were noncarriers. Both groups showed significant improvements in mean pulmonary artery pressure and pulmonary vascular resistance following BPA. However, the 6\u2010minute walk distance, symptoms, cardiac index, and right ventricular function did not significantly improve in heterozygous\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      variant carriers, whereas noncarriers showed notable improvements. Group differences in mean change from baseline to follow\u2010up were significant in cardiac index (0.4\u2009L\/min\u2009per\u2009m\n                      <jats:sup>2<\/jats:sup>\n                      [95% CI, 0.1\u20130.8];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.019), 6\u2010minute walk distance (70\u2009m [95% CI, 6\u2013135];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.036), and right ventricular ejection fraction (9% [95% CI, 5\u201312];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      &lt;0.001), all favoring noncarriers. Of the 515 BPA procedures, complications were significantly higher in variant carriers than in noncarriers (25% versus 8%;\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      &lt;0.001). Multivariate logistic regression analysis indicated a significant association between the\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant and BPA complications (adjusted odds ratio, 7.0 [95% CI, 1.1\u201344.4];\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\">\n                      Heterozygous\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant carriers exhibited a poor response to BPA, suggesting that the\n                      <jats:italic toggle=\"no\">RNF213<\/jats:italic>\n                      p.Arg4810Lys variant could be a risk factor for BPA complications.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.124.039002","type":"journal-article","created":{"date-parts":[[2025,4,16]],"date-time":"2025-04-16T23:34:16Z","timestamp":1744846456000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Balloon Pulmonary Angioplasty in Heterozygous\n                    <i>RNF213<\/i>\n                    p.Arg4810Lys Variant Carriers Diagnosed With Chronic Thromboembolic Pulmonary Hypertension"],"prefix":"10.1161","volume":"14","author":[{"given":"Takatoyo","family":"Kiko","sequence":"first","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0303-6546","authenticated-orcid":false,"given":"Ryotaro","family":"Asano","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]},{"name":"National Cerebral and Cardiovascular Center Research Institute","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1953-5016","authenticated-orcid":false,"given":"Tomohiko","family":"Ishibashi","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center Research Institute","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Endo","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-4477-2637","authenticated-orcid":false,"given":"Shinya","family":"Fujisaki","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Takano","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsumasa","family":"Akao","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0683-4423","authenticated-orcid":false,"given":"Naruhiro","family":"Nishi","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3053-0670","authenticated-orcid":false,"given":"Hiroya","family":"Hayashi","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akiyuki","family":"Kotoku","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5305-7823","authenticated-orcid":false,"given":"Hiroki","family":"Horinouchi","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3942-8499","authenticated-orcid":false,"given":"Jin","family":"Ueda","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3280-6119","authenticated-orcid":false,"given":"Akihiro","family":"Tsuji","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7579-0674","authenticated-orcid":false,"given":"Tetsuya","family":"Fukuda","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8404-2587","authenticated-orcid":false,"given":"Yoshikazu","family":"Nakaoka","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center Research Institute","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1180-9262","authenticated-orcid":false,"given":"Takeshi","family":"Ogo","sequence":"additional","affiliation":[{"name":"National Cerebral and Cardiovascular Center","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.024"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehac237"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/circoutcomes.117.004029"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02095-2018"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00012914"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2018.11.026"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0022542"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.3390\/life12040555"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1161\/strokeaha.113.002477"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2017.09.023"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2021.08.058"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1161\/jaha.124.035009"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01511-2023"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00763-2023"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2019.08.022"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/SVIN.122.000396"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2023.12.005"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejrad.2016.12.013"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejrad.2016.06.010"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1161\/circinterventions.112.971077"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1161\/circinterventions.115.003318"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2024.132343"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2022.05.002"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.2176\/nmc.52.299"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2010.132"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12199-015-0498-7"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1161\/jaha.119.011996"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmcr.2019.100977"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/s2213-2600(15)00544-5"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200908-1284OC"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.124.039002","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T10:56:28Z","timestamp":1784112988000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.124.039002"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,6]]},"references-count":30,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2025,5,6]]}},"alternative-id":["10.1161\/JAHA.124.039002"],"URL":"https:\/\/doi.org\/10.1161\/jaha.124.039002","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,5,6]]},"assertion":[{"value":"2024-09-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-30","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-16","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e039002"}}