{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T09:50:38Z","timestamp":1790070638135,"version":"4.0.1"},"reference-count":20,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2020,3,17]]},"abstract":"\n Background<\/jats:title>\n \n Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (\n SCD<\/jats:styled-content>\n ), yet the underlying causes are not well understood. We sought to define the predictors of\n SCD<\/jats:styled-content>\n in this population.\n <\/jats:p>\n <\/jats:sec>\n \n Methods and Results<\/jats:title>\n \n A retrospective analysis of all patients with Eisenmenger syndrome from 2 large tertiary referral centers was performed. ECGs, prolonged ambulatory recordings, echocardiograms, and clinical histories were reviewed; and the cause of death was identified. A total of 246 patients (85 [34.6%] men) with a mean age of 37.3 (\u00b114.2) years were followed up for a median of 7\u00a0years. Over the study period, 136 patients died, with 40 experiencing\n SCD<\/jats:styled-content>\n and 74 experiencing cardiac death (sudden and nonsudden). Age, atrial fibrillation, prolonged\n QRS<\/jats:styled-content>\n duration, complete heart block, right atrial enlargement, right bundle branch block, increased right atrial pressure, impaired biventricular function, and the presence of a pacemaker were associated with increased risk of\n SCD<\/jats:styled-content>\n , whereas advanced pulmonary hypertension therapies were protective. Atrial fibrillation (11.45\u2010fold increased risk;\n P<\/jats:italic>\n <0.001) and\n QRS<\/jats:styled-content>\n duration \u2265120\u00a0ms (2.06\u2010fold increased risk;\n P<\/jats:italic>\n =0.034) remained significant predictors of\n SCD<\/jats:styled-content>\n in the multivariate analysis, whereas advanced pulmonary hypertension therapies were strongly protective against\n SCD<\/jats:styled-content>\n (\n P<\/jats:italic>\n <0.001).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Atrial arrhythmias, impaired ventricular function, and conduction system disease were associated with increased risk of\n SCD<\/jats:styled-content>\n in this cohort of patients with Eisenmenger syndrome, providing an opportunity for early risk stratification and potential intervention. Clinical heart failure symptoms (New York Heart Association class \u2265\n II<\/jats:styled-content>\n ) were predictive of increased mortality but not of\n SCD<\/jats:styled-content>\n , suggesting a potential arrhythmic cause behind\n SCD<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1161\/jaha.119.014554","type":"journal-article","created":{"date-parts":[[2020,3,15]],"date-time":"2020-03-15T05:02:11Z","timestamp":1584248531000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":18,"title":["Determinants of Sudden Cardiac Death in Adult Patients With Eisenmenger Syndrome"],"prefix":"10.1161","volume":"9","author":[{"given":"Anca","family":"Chiriac","sequence":"first","affiliation":[{"name":"Departments of Cardiovascular Diseases Mayo Clinic Jacksonville FL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David C.","family":"Riley","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Russell","sequence":"additional","affiliation":[{"name":"Division of Cardiology Ahmanson\/UCLA Adult Congenital Heart Disease Center, UCLA Medical Center University of California Los Angeles CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy P.","family":"Moore","sequence":"additional","affiliation":[{"name":"Division of Cardiology Ahmanson\/UCLA Adult Congenital Heart Disease Center, UCLA Medical Center University of California Los Angeles CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepak","family":"Padmanabhan","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David O.","family":"Hodge","sequence":"additional","affiliation":[{"name":"Health Sciences Research Mayo Clinic Jacksonville FL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew R.","family":"Spiegel","sequence":"additional","affiliation":[{"name":"Health Sciences Research Mayo Clinic Jacksonville FL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily R.","family":"Vargas","sequence":"additional","affiliation":[{"name":"Health Sciences Research Mayo Clinic Jacksonville FL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina D.","family":"Phillips","sequence":"additional","affiliation":[{"name":"Departments of Cardiovascular Diseases Mayo Clinic Jacksonville FL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naser M.","family":"Ammash","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malini","family":"Madhavan","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel J.","family":"Asirvatham","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher J.","family":"McLeod","sequence":"additional","affiliation":[{"name":"Departments of Cardiovascular Diseases Mayo Clinic Jacksonville FL"},{"name":"Department of Cardiovascular Diseases Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2008.11.025"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehx201"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.04.018"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1606222"},{"key":"e_1_3_2_6_2","volume-title":"Diagnosis and Management of Adult Congenital Heart Disease","author":"Gatzoulis MA","year":"2018","unstructured":"Gatzoulis MA, Webb GD, Daubeney PEF. 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