Display options
Share it on

Open Heart. 2016 Jul 11;3(2):e000407. doi: 10.1136/openhrt-2016-000407. eCollection 2016.

Sudden cardiac death in adults with congenitally corrected transposition of the great arteries.

Open heart

A McCombe, F Touma, D Jackson, C Canniffe, P Choudhary, L Pressley, D Tanous, Peter J Robinson, D Celermajer

Affiliations

  1. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Discipline of Medicine, Central Clinical School, University of Sydney, Sydney, New South Wales, Australia.
  2. Department of Cardiology , Royal Prince Alfred Hospital , Sydney, New South Wales , Australia.
  3. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Department of Cardiology, Mater Misericordiae Hospital, Dublin, Ireland.
  4. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Discipline of Medicine, Central Clinical School, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Westmead Hospital, Sydney, New South Wales, Australia.

PMID: 27493760 PMCID: PMC4947757 DOI: 10.1136/openhrt-2016-000407

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.

Keywords: Cardiac arrhythmias and resuscitation science; Complex congenital heart disease

References

  1. Circulation. 1998 Sep 8;98(10):997-1005 - PubMed
  2. Circulation. 2012 Oct 16;126(16):1944-54 - PubMed
  3. Pediatr Cardiol. 1999 Nov-Dec;20(6):411-7 - PubMed
  4. Hum Pathol. 1995 Oct;26(10):1065-72 - PubMed
  5. Am J Epidemiol. 1985 Jan;121(1):31-6 - PubMed
  6. Can J Cardiol. 1996 May;12(5):526-8 - PubMed
  7. Pediatrics. 1980 Feb;65(2 Pt 2):375-461 - PubMed
  8. Indian Pacing Electrophysiol J. 2010 Apr 01;10(4):179-83 - PubMed
  9. J Interv Card Electrophysiol. 2004 Dec;11(3):211-5 - PubMed
  10. Eur Heart J. 2010 Dec;31(23):2915-57 - PubMed
  11. Am J Cardiol. 2000 Nov 15;86(10):1111-6 - PubMed
  12. Can J Cardiol. 1996 Nov;12(11):1161-3 - PubMed
  13. Am J Cardiol. 2011 Sep 1;108(5):729-34 - PubMed
  14. J Am Coll Cardiol. 2004 Sep 1;44(5):1095-102 - PubMed
  15. Am J Cardiol. 1986 Aug 1;58(3):319-24 - PubMed
  16. Circulation. 2002 Mar 12;105(10 ):1189-94 - PubMed
  17. Eur Heart J. 2010 May;31(10):1220-9 - PubMed
  18. J Invasive Cardiol. 2007 May;19(5):E139-41 - PubMed
  19. J Am Coll Cardiol. 1996 Apr;27(5):1238-43 - PubMed
  20. J Am Coll Cardiol. 2013 Sep 3;62(10 ):926-36 - PubMed
  21. J Am Coll Cardiol. 2000 Jul;36(1):255-61 - PubMed
  22. Circulation. 2006 Dec 12;114(24):2699-709 - PubMed
  23. Arch Mal Coeur Vaiss. 1979 Jan;72(1):113-7 - PubMed

Publication Types