Cerebrovascular accidents in Ebstein’s anomaly

Nicholas Y. Tan, Christine H. Attenhofer Jost, Murray D. Polkinghorne, Emily R. Vargas, David O. Hodge, Joseph A. Dearani, Samuel J. Asirvatham, Heidi M. Connolly, Christopher J. McLeod

Research output: Contribution to journalArticle

Abstract

Introduction: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein’s anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients. Methods: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method. Results: Nine hundred sixty-eight patients (median age 21.1 years, 41.5% male) were included, in which, 87 patients (9.0%) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95% CI 2.60-21.22; p =.0002) and migraines/headaches (OR 2.38; 95% CI 1.40-4.04; p =.0013) but not atrial arrhythmias (OR 0.75; 95% CI 0.44-1.30; p =.31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p =.076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4%, 15.9%, and 23.5%, respectively, with paradoxical embolism heavily implicated. Conclusion: Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.

Original languageEnglish (US)
JournalCongenital Heart Disease
DOIs
StateAccepted/In press - Jan 1 2019

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Ebstein Anomaly
Stroke
Odds Ratio
Migraine Disorders
Cardiac Arrhythmias
Paradoxical Embolism
Patent Foramen Ovale
Brain Abscess
Tricuspid Valve
Atrial Heart Septal Defects
Transient Ischemic Attack
Incidence

Keywords

  • cerebrovascular accident
  • Ebstein’s anomaly
  • stroke

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Tan, N. Y., Attenhofer Jost, C. H., Polkinghorne, M. D., Vargas, E. R., Hodge, D. O., Dearani, J. A., ... McLeod, C. J. (Accepted/In press). Cerebrovascular accidents in Ebstein’s anomaly. Congenital Heart Disease. https://doi.org/10.1111/chd.12841

Cerebrovascular accidents in Ebstein’s anomaly. / Tan, Nicholas Y.; Attenhofer Jost, Christine H.; Polkinghorne, Murray D.; Vargas, Emily R.; Hodge, David O.; Dearani, Joseph A.; Asirvatham, Samuel J.; Connolly, Heidi M.; McLeod, Christopher J.

In: Congenital Heart Disease, 01.01.2019.

Research output: Contribution to journalArticle

Tan, NY, Attenhofer Jost, CH, Polkinghorne, MD, Vargas, ER, Hodge, DO, Dearani, JA, Asirvatham, SJ, Connolly, HM & McLeod, CJ 2019, 'Cerebrovascular accidents in Ebstein’s anomaly', Congenital Heart Disease. https://doi.org/10.1111/chd.12841
Tan NY, Attenhofer Jost CH, Polkinghorne MD, Vargas ER, Hodge DO, Dearani JA et al. Cerebrovascular accidents in Ebstein’s anomaly. Congenital Heart Disease. 2019 Jan 1. https://doi.org/10.1111/chd.12841
Tan, Nicholas Y. ; Attenhofer Jost, Christine H. ; Polkinghorne, Murray D. ; Vargas, Emily R. ; Hodge, David O. ; Dearani, Joseph A. ; Asirvatham, Samuel J. ; Connolly, Heidi M. ; McLeod, Christopher J. / Cerebrovascular accidents in Ebstein’s anomaly. In: Congenital Heart Disease. 2019.
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abstract = "Introduction: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein’s anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients. Methods: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method. Results: Nine hundred sixty-eight patients (median age 21.1 years, 41.5{\%} male) were included, in which, 87 patients (9.0{\%}) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95{\%} CI 2.60-21.22; p =.0002) and migraines/headaches (OR 2.38; 95{\%} CI 1.40-4.04; p =.0013) but not atrial arrhythmias (OR 0.75; 95{\%} CI 0.44-1.30; p =.31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p =.076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4{\%}, 15.9{\%}, and 23.5{\%}, respectively, with paradoxical embolism heavily implicated. Conclusion: Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.",
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AU - Hodge, David O.

AU - Dearani, Joseph A.

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