Congenital Heart Surgeons' Society
CHSS Home CHSS Home Past & Future Meetings Past & Future Meetings

Back to 2023 Abstracts


Natural History Of Congenitally Corrected Transposition Of The Great Arteries: An International Multicentre Study.
Edward Buratto, MD, PhD1, Emile A. Bacha, MD1, Emre Belli2, Paul Kurlansky, MD1, David J. Barron, MD3, Mohamed Ly, MD4, Lynne Nield, MD5, Christopher McLeod, MD6, Sonya Babu-Narayan, MD7, Alban-Elouen Baruteau, MD4, David M. Kalfa, MD1.
1Columbia University, New York, NY, USA, 2Centre Chirurgical Marie Lannelongue, Paris, France, 3The Hospital For Sick Children, Toronto, ON, Canada, 4CHU Nantes, Nantes, France, 5The Hospital for Sick Children, Toronto, ON, Canada, 6Mayo Clinic, Rochester, MN, USA, 7Royal Brompton Hospital, London, United Kingdom.

Objective(s):
The optimal management of patients with congenitally corrected transposition of the great arteries (ccTGA) remains unknown. Although anatomic correction has been advocated due to the risk of systemic right ventricular dysfunction, patients may survive into their 7th decade without surgery. We conducted a multicentre study to investigate the natural history of patients with ccTGA.
Methods:
A multicentre, international, retrospective study was conducted in 29 hospitals in 6 countries from 1990 to 2018. Patients with ccTGA who did not undergo surgery were included. Comparison of survival to an age- and sex-matched general population was performed. Results:
Of a total of 362 patients, 68.5% (248/362) had isolated ccTGA while 31.5% (114/362) had ccTGA with ventricular septal defect (VSD) and/or pulmonary stenosis (PS). Survival at 30 and 70 years was 95.26.4% and 53.521.1% for patients with isolated ccTGA, 93.38.2% and 80.019.4% for patients with ccTGA/PS/VSD. The independent predictor of mortality for patients with isolated ccTGA was left ventricular dysfunction (HR=3.8, p=0.01).For patients with isolated ccTGA the ratio of observed/expected survival at 70-years was 0.51 (95%CI: 0.19-0.83), suggesting a significantly lower than expected survival. For patients with ccTGA with VSD/PS the ratio of observed/expected survival was 0.96 (95%CI: 0.49-1.2), suggesting equivalent survival to the matched population. Conclusions:
Survival of patients with isolated ccTGA was significantly lower than a matched population at 70-years, with left ventricular dysfunction at presentation independently predicting mortality. Conversely, patients with ccTGA/VSD/PS with balanced physiology who did not require surgery have similar survival to the matched population up to 70-years.


Back to 2023 Abstracts