Single ventricle palliation in congenitally corrected transposition of the great arteries: an international multicentre study.

BACKGROUND

Single ventricle palliation (SVP) may be performed in patients with congenitally corrected transposition of the great arteries (ccTGA) due to either hypoplasia of one ventricle, or anatomic complexity rendering biventricular repair unfeasible. There have been only a few, small studies of the outcomes of SVP in the setting of ccTGA.

METHODS

A multicentre, international, retrospective cohort study of patients with ccTGA undergoing SVP was conducted in 29 tertiary hospitals in 6 countries from 1990 to 2018.

RESULTS

A total of 194 patients with ccTGA underwent SVP. A functionally univentricular heart was present in 62.4% (121/194). Patients with two good-sized ventricles had more laterality defects (50.7% v 28.1%, p=0.002). Fontan completion was achieved in 80.3% (155/194). A tricuspid valve surgery was performed in 9.3% (18/194). Survival was 97.4% (95% CI: 92.1-99.2%) at 15 years in the univentricular cohort, while it was 89.8% (95% CI: 78.0-95.5%) at 15 years in those with two adequate ventricles, p = 0.05. At last follow-up 11.5% of patients had heart failure, while 6.0% had moderate or greater systemic RV dysfunction and 8.4% exhibited moderate or greater tricuspid regurgitation. In multivariable analysis, aortic coarctation or hypoplasia (HR=7.7, p=0.005) was associated with mortality.

CONCLUSIONS

Single ventricle palliation in patients with ccTGA is associated with excellent long-term survival and low rates of heart failure and atrioventricular valve failure. In patients who would require complex surgery to achieve a biventricular repair, SVP appears to be a good alternative.

Informations de copyright

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