Emerging Roles for Cardiovascular Magnetic Resonance in Adult Congenital Heart Disease Electrophysiology

  • Sonya V. Babu-NarayanEmail author
  • Jennifer Keegan
  • Sabine I. S. Ernst


The population of adults with congenital heart disease is growing. Most children with congenital heart disease now survive into adulthood thanks to innovations in cardiac surgery that allow repair or palliation of many anatomical defects. However, arrhythmia and sudden death remain important causes of late morbidity and mortality. As the population of adult congenital heart disease (ACHD) ages there is an increasing burden of arrhythmia requiring specialist care. Rhythm disturbances may be the first presenting symptom and herald a need for surgical or transcatheter reintervention in which case treatment should be directed at the underlying cardiovascular hemodynamics. In this case electrophysiological intervention is a secondary concern. Arrhythmia and sudden cardiac death may, alternatively occur in the absence of a target hemodynamic lesion. Arrhythmia mechanisms vary according to the exact underlying anatomic congenital defect and method and timing of surgical repair. Whilst the arrhythmia can relate to underlying structural heart disease, such as Wolff-Parkinson-White syndrome associated with Ebstein’s anomaly, it often relates to surgically acquired scars combined with chamber enlargement as a consequence of abnormal pressure and volume loads. In congenital heart disease, atrial arrhythmias frequently emanate from the right atrium or right ventricle and are not generally confined to the left atrium as with atrial fibrillation triggered by pulmonary vein muscle bundles. ACHD patients with atrial arrhythmia are at particular risk of tachycardia induced cardiomyopathy and existing hemodynamic lesions such as valvular regurgitation may be exacerbated by arrhythmia. There is also overlap in clinical presentations, such that ACHD patients presenting with atrial arrhythmia are at higher risk of ventricular arrhythmia [1]. Atrial arrhythmia is well recognized as an important indicator of ventricular dysfunction in congenital heart disease [2]. Adults with congenital heart disease are at risk of sudden cardiac death and may be referred for diagnostic electrophysiological (EP) ventricular stimulation study to aid risk stratification [1, 3, 4]. Both atrial and ventricular stimulation at EP study may therefore be indicated for the same patient.


Congenital Heart Disease Pulmonary Vein Late Gadolinium Enhancement Atrial Arrhythmia Atrial Fibrillation Ablation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Conflicts of Interest

SE is a consultant for Stereotaxis, Inc. and Biosense Webster.

SVBN is supported by a British Heart Foundation Fellowship. The work of the CMR unit is supported by the NIHR Cardiovascular Biomedical Research Unit of Royal Brompton and Harefield NHS Foundation Trust and Imperial College London.

Supplementary material

218193_1_En_19_MOESM1_ESM.avi (25.5 mb)
Movie 19.1 3D bSSFP for 3D volume reconstruction and image integration with 3D EP mapping systems to facilitate retrograde approach and avoid puncture of baffled atrial pathways after Mustard operation for transposition of the great arteries. This patient followed up after Mustard operation for transposition of the great arteries presented with atrial tachycardia requiring cardioversion. His CMR study was performed in sinus rhythm and 3D bSSFP was timed in systole. (a) 3D bSSFP imaging was performed and subsequently segmented with CARTO. The subaortic (red) heavily trabeculated RV (purple) is shown. The pulmonary arteries are colored orange and the subpulmonary LV brighter purple. The systemic venous baffled atrial compartment (turquoise) and pulmonary venous compartment (yellow) were separately segmented. A rotating movie can be seen. (b) 3D CMR reconstructions of the systemic venous compartment (SVA) and pulmonary venous atrial compartment (PVA) were displayed and merged to the local activation time map. The CMR roadmap of all the heart chambers was merged with the EP maps and displayed superimposed on fluoroscopy. (c) Low voltage (grey) scar was found in the pulmonary venous compartment. The dark red tags depict the ablation sites. (d) This site in the left inferior pulmonary vein was reached using retrograde access from the femoral artery and avoiding puncture of the surgical baffle. Retrograde manipulation was made possible by the combination of CMR image integration and remote navigation EP using magnetic navigation allowing several S bends of the catheter. The “MAP” catheter is shown and the retrograde path to reach the site of ablation can be appreciated on the fluoroscopy image. The other two catheters are in the LV apex (LVA) and systemic venous atrial appendage (SVAA) respectively (AVI 26064 kb)
218193_1_En_19_MOESM2_ESM.avi (21.7 mb)
Movie 19.2 3D bSSFP for 3D volume reconstruction and image integration with 3D EP mapping systems to facilitate retrograde approach and avoid puncture of lateral tunnel total cavopulmonary connection and integrated EP activation map. A patient with single ventricle physiology presented clinically with recurrent atrial tachycardia status post lateral tunnel total cavopulmonary connection (TCPC, blue). The TCPC was performed for situs solitus, discordant atrioventricular connections, double inlet left ventricle, transposed great arteries and pulmonary stenosis. The CMR was performed in established atrial arrhythmia and 3D bSSFP was timed in systole. (a) 3D reconstruction of 3D bSSFP CMR imaging showing the lateral tunnel total cavopulmonary connection (turquoise) and the remaining native RA (yellow). The underlying anatomy was double inlet LV (purple). A rotating whole heart structure can be seen in the attached movie whereby the aorta is red, and left atrium dark blue. (b) In this image the ventricular mass has been masked. Re-entrant tachycardia was mapped in the residual RA with counterclockwise activation around the tricuspid annulus. (c) There is bystander activation of the left atrium (AVI 22225 kb)


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Copyright information

© Springer-Verlag London 2012

Authors and Affiliations

  • Sonya V. Babu-Narayan
    • 1
    • 2
    Email author
  • Jennifer Keegan
    • 2
  • Sabine I. S. Ernst
    • 2
  1. 1.NIHR Cardiovascular Biomedical Research UnitRoyal Brompton and Harefield NHS Foundation TrustLondonUK
  2. 2.National Heart and Lung Institute, Imperial CollegeLondonUK

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