PMID- 27493760 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160805 LR - 20210112 IS - 2053-3624 (Print) IS - 2053-3624 (Electronic) IS - 2053-3624 (Linking) VI - 3 IP - 2 DP - 2016 TI - Sudden cardiac death in adults with congenitally corrected transposition of the great arteries. PG - e000407 LID - 10.1136/openhrt-2016-000407 [doi] LID - e000407 AB - 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 ( approximately 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. FAU - McCombe, A AU - McCombe A AD - 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. FAU - Touma, F AU - Touma F AD - 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. FAU - Jackson, D AU - Jackson D AD - Department of Cardiology , Royal Prince Alfred Hospital , Sydney, New South Wales , Australia. FAU - Canniffe, C AU - Canniffe C AD - Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Department of Cardiology, Mater Misericordiae Hospital, Dublin, Ireland. FAU - Choudhary, P AU - Choudhary P AD - 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. FAU - Pressley, L AU - Pressley L AD - 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. FAU - Tanous, D AU - Tanous D AD - 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. FAU - Robinson, Peter J AU - Robinson PJ AD - 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. FAU - Celermajer, D AU - Celermajer D AD - 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. LA - eng PT - Journal Article DEP - 20160711 PL - England TA - Open Heart JT - Open heart JID - 101631219 PMC - PMC4947757 OTO - NOTNLM OT - Cardiac arrhythmias and resuscitation science OT - Complex congenital heart disease EDAT- 2016/08/06 06:00 MHDA- 2016/08/06 06:01 PMCR- 2016/07/11 CRDT- 2016/08/06 06:00 PHST- 2016/01/11 00:00 [received] PHST- 2016/04/30 00:00 [revised] PHST- 2016/05/24 00:00 [accepted] PHST- 2016/08/06 06:00 [entrez] PHST- 2016/08/06 06:00 [pubmed] PHST- 2016/08/06 06:01 [medline] PHST- 2016/07/11 00:00 [pmc-release] AID - openhrt-2016-000407 [pii] AID - 10.1136/openhrt-2016-000407 [doi] PST - epublish SO - Open Heart. 2016 Jul 11;3(2):e000407. doi: 10.1136/openhrt-2016-000407. eCollection 2016.