@article {McCombee000407, author = {A McCombe and F Touma and D Jackson and C Canniffe and P Choudhary and L Pressley and D Tanous and Peter J Robinson and D Celermajer}, title = {Sudden cardiac death in adults with congenitally corrected transposition of the great arteries}, volume = {3}, number = {2}, elocation-id = {e000407}, year = {2016}, doi = {10.1136/openhrt-2016-000407}, publisher = {Archives of Disease in childhood}, 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 {\textquoteleft}two-ventricle{\textquoteright} circulations. 65\% were male. The mean age at diagnosis was 12.4{\textpm}11.4 years and the mean age at last time of review was 34.3{\textpm}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{\textendash}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.}, URL = {https://openheart.bmj.com/content/3/2/e000407}, eprint = {https://openheart.bmj.com/content/3/2/e000407.full.pdf}, journal = {Open Heart} }