Pulmonary Vein-Triggered Atrial Fibrillation in Unrepaired Double-Outlet Right Ventricle With Pulmonary Atresia.

Figures

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  Figure 1. 12-Lead Electrocardiograms (A) Atrial fibrillation with a mean ventricular rate of 78 beats/min. (B) Sinus rhythm with a mean ventricular rate of 72 beats/min.

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  Figure 2. 3-Dimensional Images of the Cardiac Anatomy (A) The great arteries, cardiac chambers, and vena cava. The main and left PAs are absent. The right PA is perfused via a modified BT shunt. The aorta arises from the RV, and there are no great arteries in fibrous continuity with the mitral valve. (B) The atria, ventricle, and vena cava. The right common PV is compressed by the descending aorta. BT = Blalock-Taussig; LA = left atrium; LAA = left atrial appendage; LIPV = left inferior pulmonary vein; LSPV = left superior pulmonary vein; LV = left ventricle; PA = pulmonary artery; PV = pulmonary vein; RA = right atrium; RAA = right atrial appendage; RV = right ventricle; SVC = superior vena cava.

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  Figure 3. Electrophysiological Data and Catheter Ablation (A) The mapping catheter is positioned in the LSPV when the ectopy is observed. (B) Ectopic beat from the LSPV induces atrial fibrillation. The amplitude of the earliest activation site of the ectopic beat (magenta arrow), recorded at the tip electrodes (Pentaray 1-2), is lower than that observed during sinus rhythm. (C) The lesion of PV isolation. (D) Postprocedural activation map inside the right pulmonary vein. The earliest activation site is inside the right pulmonary vein. CS = coronary sinus; LAT = local activation time; other abbreviations as in Figure 2.

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  Visual Summary. Pulmonary Vein–Triggered Atrial Fibrillation in Unrepaired Double-Outlet Right Ventricle With Pulmonary Atresia Pulmonary vein–triggered AF was identified in a patient with unrepaired DORV, pulmonary atresia, and juxtaposition of the atrial appendages. Radiofrequency ablation with PVI achieved clinical success, highlighting the relevance of an empirical PV-based ablation strategy. ACHD = adult congenital heart disease; AF = atrial fibrillation; CHD = congenital heart disease; CS = coronary sinus; d = distal; DORV = double-outlet right ventricle; LAA = left atrial appendage; LSPV = left superior pulmonary vein; LV = left ventricle; p = proximal; PA = pulmonary artery; PV = pulmonary vein; PVI = pulmonary vein isolation; RA = right atrium; RAA = right atrial appendage; RV = right ventricle.