AP Target Symposium 3 (II-APT3)
Dealing with congenitally corrected transposition of the great arteries - Efforts to minimize late development of systemic ventricular dysfunction

[II-APT3-02] The late outcome of systemic right ventricle in congenitally corrected transposition of great arteries: Functional repair or anatomical repair

^○Hajime Ichikawa¹, Takaya Hoashi¹, Tomohiro Nakata¹, Masatoshi Shimada¹, Akihiko Higashida¹, Hideo Ohuchi², Kenichi Kurosaki², Isao Shiraishi² (1.Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Japan, 2.Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Japan)

The poor outcome of systemic right ventricle (RV) in congenitally corrected transposition of great arteries (ccTGA) is a well-known fact. Our strategy for the treatment of ccTGA is to place the left ventricle in a systemic position since 1990. The outcome is compared with the functional repair. Patients and method: One hundred sixty one ccTGA patients with balanced ventricle were included in this retrospective non-randomized analysis. There were 81 patients with conventional repair including simple VSD closure or complex LV-PA conduit with interventricular rerouting. Seventy patients underwent double switch operation of 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 1.8 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.