Clinical Factors Affecting Survival in Patients with Congenitally Corrected Transposition of the Great Arteries: A Systematic Review and Meta-Analysis

Background: Congenitally corrected transposition of the great arteries (cc-TGA) is a defect characterized by arterio-ventricular and atrioventricular disconcordance. Most patients have co-existing cardiac abnormalities that warrant further treatment. Some patients do not require surgical intervention, but most undergo physiological repair or anatomical surgery, which enables them to reach adulthood. Aims: We aimed to evaluate mortality risk factors in patients with cc-TGA. Results: We searched the PubMed database and included 10 retrospective cohort studies with at least a 5-year follow-up time with an end-point of cardiovascular death a minimum of 30 days after surgery. We enrolled 532 patients, and 83 met the end-point of cardiovascular death or equivalent event. As a risk factor for long-term mortality, we identified New York Heart Association (NYHA) class >= III/heart failure hospitalization (OR = 10.53; 95% CI, 3.17-34.98) and systemic ventricle dysfunction (SVD; OR = 4.95; 95% CI, 2.55-9.64). We did not show history of supraventricular arrhythmia (OR = 2.78; 95% CI, 0.94-8.24), systemic valve regurgitation >= moderate (SVR; OR = 4.02; 95% Cl, 0.84-19.18), and pacemaker implantation (OR = 1.48; 95% Cl, 0.12-18.82) to affect the long-term survival. In operated patients only, SVD (OR = 4.69; 95% CI, 2.06-10.71) and SVR (OR = 3.85; 95% CI, 1.5-9.85) showed a statistically significant impact on survival. Conclusions: The risk factors for long-term mortality for the entire cc-TGA population are NYHA class >= III/heart failure hospitalization and systemic ventricle dysfunction. In operated patients, systemic ventricle dysfunction and at least moderate systemic valve regurgitation were found to affect survival.