Sudden cardiac death in adults with congenitally corrected transposition of the great arteries.

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.

Late outcomes in adults with coarctation of the aorta.

AIMS: Previous cohort studies of patients with coarctation of the aorta (CoA) demonstrate reduced long-term survival. Improved surgical outcomes in children and evolution of adult congenital heart disease (ACHD) services have resulted in improved survival in patients with other CHDs. We hypothesise that for young adult patients with CoA long-term outcomes have improved in the contemporary era. METHODS: 151 patients (58% men) with simple CoA followed up at a tertiary ACHD service in Sydney, Australia, from 1993 to 2013 were included. We documented mortality and major morbidity such as the need for re-intervention for re-coarctation or aneurysms. RESULTS: 140 patients (mean age 35±15 years) underwent CoA repair at median age of 5 (IQR 0-10) years. Initial surgical strategy included end-to-end repair in 43, subclavian flap aortoplasty in 28 and patch aortoplasty in 31 patients (and was not documented in 28 cases). 6 patients had endovascular repair, 4 had interposition tube grafts and 11 were unrepaired. There were a total of seven deaths at a median age of 60 years. Actuarial survival was 98% at 40, 98% at 50 and 89% at 60 years of age. Re-coarctation occurred in 34% and descending aortic aneurysms were noted in 18%. Patients with end-to-end repair had lower rates of significant re-coarctation or descending aortic aneurysms (p=0.026 and <0.001, respectively). 66% had bicuspid aortic valve and 44% were hypertensive. CONCLUSIONS: Patients with CoA who reach adolescence demonstrate very good long-term survival up to age 60 years. Long-term morbidity is common, however, related largely to aortic complications and late hypertension.