Abstract 18479: Double Outlet Right Ventricle in the Adult: A Broad Spectrum of Anatomic Diagnoses and Their Association With Arrhythmia and Sudden Death
Background: Double outlet right ventricle (DORV) includes a broad spectrum of anatomic diagnoses, most of which amenable to biventricular repair, with a resultant improvement in survival. Limited data exist on the incidence of arrhythmia and sudden cardiac death (SCD) in adults with DORV.
Methods: Adult DORV patients (pts).followed at a tertiary center (1999-2016) were included and grouped into: group 1 (subaortic VSD type), group 2 (Fallot-type), group 3 (transposition with pulmonary stenosis), group 4 (transposition without pulmonary stenosis) and group 5 (complex anatomy). The primary endpoint was the composite of all-cause mortality and appropriate Implantable Cardioverter Defibrillator (ICD) shocks. The composite endpoint of SCD and appropriate ICD shocks was the secondary endpoint.
Results: Overall, 151 pts were included: 34 (22.5%) in group 1, 23 (15.2%) in group 2, 26 (17.2%) in group 3, 16 (10.6%) in group 4 and 52 (34.4%) in group 5. Over a median follow up period of 7.5 years, supraventricular tachycardias (SVTs) were observed during follow-up in 35 pts (3.2% per person years(ppy)), the most frequent being atrial tachycardia (n=23) (2.1% ppy). At least one ventricular tachycardia (VT) was seen in 15 pts (1.3% ppy). During this period, 29 pts died (mortality rate 2.3% ppy). There were 7 cases of SCD and 3 appropriate ICD shocks. Other deaths were mainly heart failure related. QRS duration >180msec at baseline (HR 7.5, 95%CI:2.2-26.1, p=0.002), history of SVT (HR 8.8, 95%CI:2.9-26.7, p<0.001) and prior palliative operations (HR 3.7, 95%CI:1.6-8.2, p=0.002) were significant predictors of the primary endpoint. QRS duration>180ms (HR 21.2, 95%CI:5.3-85.7, p<0.001) and SVT history (HR 9.6, 95%CI:1.9-46.8, p=0.005) were significant predictors of the composite secondary endpoint.
Conclusion: Arrhythmias and SCD are not uncommon in patients with DORV. Risk stratification tools are required to identify patients at risk who should be followed closely and, if at risk of SCD, offered an ICD for primary prevention.
Author Disclosures: M. Boutsikou: Research Grant; Modest; Hellenic Society of Cardiology. A. Silveira-Correa: None. A. Kempny: None. M. Grubler: None. P. Piatek: None. R. Alonso-Gonzalez: None. L. Swan: None. A. Uebing: None. M.A. Gatzoulis: None. K. Dimopoulos: None.
- © 2016 by American Heart Association, Inc.