title: Sudden cardiac death in adults with congenitally corrected transposition of the great arteries.
abstract: 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.
  
  
  
  A retrospective review of the medical records of all patients attending our adult congenital heart centre, with known ccTGA.
  
  
  
  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.
  
  
  
  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.
authors: 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.
authors: A
authors: A
authors: McCombe
authors: 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.
authors: F
authors: F
authors: Touma
authors: Department of Cardiology , Royal Prince Alfred Hospital , Sydney, New South Wales , Australia.
authors: D
authors: D
authors: Jackson
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Department of Cardiology, Mater Misericordiae Hospital, Dublin, Ireland.
authors: C
authors: C
authors: Canniffe
authors: 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.
authors: P
authors: P
authors: Choudhary
authors: 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.
authors: L
authors: L
authors: Pressley
authors: 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.
authors: D
authors: D
authors: Tanous
authors: 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.
authors: Peter J
authors: PJ
authors: Robinson
authors: 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.
authors: D
authors: D
authors: Celermajer
keywords: Cardiac arrhythmias and resuscitation science; Complex congenital heart disease