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Original research article
Sudden cardiac death in adults with congenitally corrected transposition of the great arteries
  1. A McCombe1,2,
  2. F Touma1,2,
  3. D Jackson1,
  4. C Canniffe1,3,
  5. P Choudhary1,2,4,
  6. L Pressley1,2,
  7. D Tanous1,2,4,
  8. Peter J Robinson1,2 and
  9. D Celermajer1,2
  1. 1Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  2. 2Discipline of Medicine, Central Clinical School, University of Sydney, Sydney, New South Wales, Australia
  3. 3Department of Cardiology, Mater Misericordiae Hospital, Dublin, Ireland
  4. 4Department of Cardiology, Westmead Hospital, Sydney, New South Wales, Australia
  1. Correspondence to Professor David Celermajer; david.celermajer{at}email.cs.nsw.gov.au

Abstract

Background Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart disease. There have been only few reports of sudden cardiac death (SCD) in patients with ccTGA and reasonable ventricular function.

Methods A retrospective review of the medical records of all patients attending our adult congenital heart centre, with known ccTGA.

Results From a database of over 3500 adult patients with congenital heart disease, we identified 39 (∼1%) with ccTGA and ‘two-ventricle’ circulations. 65% were male. The mean age at diagnosis was 12.4±11.4 years and the mean age at last time of review was 34.3±11.3 years. 24 patients (56%) had a history of surgical intervention. 8 (19%) had had pacemaker implantation and 2 had had a defibrillator implanted for non-sustained ventricular tachycardia (NSVT). In 544 years of patient follow-up, there had been five cases of SCD in our population; 1 death per 109 patient-years. Two of these patients had had previously documented supraventricular or NSVT. However, they were all classified as New York Heart Association (NYHA) class I or II, and systemic (right) ventricular function had been recorded as normal, mildly or mildly–moderately impaired, at most recent follow-up.

Conclusions Our experience suggests the need for improved risk stratification and/or surveillance for malignant arrhythmia in adults with ccTGA, even in those with reasonable functional class on ventricular function.

  • Cardiac arrhythmias and resuscitation science
  • Complex congenital heart disease

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

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