TY - JOUR T1 - Sudden cardiac death in adults with congenitally corrected transposition of the great arteries JF - Open Heart JO - Open Heart DO - 10.1136/openhrt-2016-000407 VL - 3 IS - 2 SP - e000407 AU - A McCombe AU - F Touma AU - D Jackson AU - C Canniffe AU - P Choudhary AU - L Pressley AU - D Tanous AU - Peter J Robinson AU - D Celermajer Y1 - 2016/07/01 UR - http://openheart.bmj.com/content/3/2/e000407.abstract N2 - 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. ER -