RT Journal Article
SR Electronic
T1 Sudden cardiac death in adults with congenitally corrected transposition of the great arteries
JF Open Heart
JO Open Heart
FD British Cardiovascular Society
SP e000407
DO 10.1136/openhrt-2016-000407
VO 3
IS 2
A1 A McCombe
A1 F Touma
A1 D Jackson
A1 C Canniffe
A1 P Choudhary
A1 L Pressley
A1 D Tanous
A1 Peter J Robinson
A1 D Celermajer
YR 2016
UL http://openheart.bmj.com/content/3/2/e000407.abstract
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 (∼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.