The 54th Annual Meeting of Japanese Society of Pediatric Cardiology and Cardiac Surgery

Presentation information

AEPC-JSPCCS Joint Symposium

AEPC-JSPCCS Joint Symposium(II-AEPCJS)
Congenitally corrected TGA

Fri. Jul 6, 2018 8:40 AM - 10:10 AM 第2会場 (301)

座長:坂本 喜三郎(静岡県立こども病院心臓血管外科)
座長:Gurleen Sharland(Evelina Children's Hospital)

[II-AEPCJS-04] Double switch operation for atrioventricular discordance with balanced ventricle

Hajime Ichikawa, Takaya Hoashi, Tomohiro Nakata, Masatoshi Shimada, Motoki Komori (Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center)

Keywords:congenitally corrected transposition of great arteries, double switch operation, atrioventricular discordance

The long-term outcome of the patients with ccTGA has been thought to be affected by the anatomical right ventricular volume overload and tricuspid regurgitation. Although it is almost a common concept that the anatomical repair is ideal, the database of Japanese Society for Thoracic Surgery reports that since 2011, the number of double switch operations (DSO) for ccTGA has been between 10 to 20 per year. On the other hand, physiological or any other procedure for ccTGA is around 100 per year in Japan. This is partly because of the fact that only a small fraction of the ccTGA cohort is suitable for DSO or the physician still hesitate to put the patients on DSO strategy which is a high risk procedure. In the presentation, we revisit the detailed indication for DSO.
There were 81 patients with conventional repair including simple VSD closure or complex LV-PA conduit with interventricular rerouting. DSO was performed in 73 patients with either Senning/Mustard plus Jatene/Rastelli type operation. Ten patients underwent only pacemaker implantation or palliative surgery. Results: Survival rates in the conventional group were poor with 10, 20 and 30 year freedom from death after surgery of 75, 71 and 65%, respectively. The age at initial surgical intervention inversely correlated with the survival (expired 9±16 vs survived 19±20). The survival rate of simple tricuspid valve replacement in 22 patients (average 29 years old) were 91, 91 and 91% in 10, 20 and 30 years after the operation, respectively. Since 1997, the survival rates after double switch operation were 97 and 91.3% at 10 and 20 years, respectively. The median age at the initial surgical intervention was 2.1 year old. Conclusion: When earlier surgical intervention is needed, conventional repair only provide poor outcome. Earlier decision of treatment strategy may improve the clinical outcome of patients with ccTGA.