Increased risk of possible paradoxical embolic events in adults with ebstein anomaly and severe tricuspid regurgitation.

Objective: Determine incidence and risk factors for possible paradoxical embolic events in patients who have Ebstein anomaly with severe tricuspid regurgitation.

Design: Retrospective study of clinical and imaging data.

Setting: Tertiary care center.

Patients: Patients undergoing clinical evaluation and echocardiography prior to cardiac surgery for Ebstein anomaly (1975-2010) performed at age ≥ 40 years.

Results: Mean age of 128 patients (81 female) was 53 ± 9 years. All had severe tricuspid regurgitation. Twenty-four (19%) had previous cardiac surgery (at <40 years), including 17 for interatrial shunt closure. Most (112 [88%]) had New York Heart Association functional class III/IV heart failure; 84 (66%) had interatrial shunting (58 had an atrial septal defect and 29 had a patent foramen ovale [3 had both]). During their lifetime, 29 patients (23%) had a history of ≥1 possible paradoxical embolic events (stroke or transient ischemic attack, brain abscess, or myocardial infarction). The best predictors of preoperative possible paradoxical embolic events were an atrial septal defect (P = .002) and older age at surgery (P = .007). There was no association of possible paradoxical embolic events with cardiovascular risk factors (hypertension, dyslipidemia, smoking, or family history of coronary artery disease) (all P ≥ .3) or atrial fibrillation (P = .69). Median age at occurrence of paradoxical embolism was 49 (range, 1.5-74 years).

Conclusions: Possible paradoxical embolic events are common in adults with Ebstein anomaly and severe tricuspid regurgitation and are strongly associated with atrial septal defect. In patients with atrial septal defect or patent foramen ovale, shunt closure should be considered to reduce risk of possible paradoxical embolic events.