1 research outputs found
Surgical Outcomes in Syndromic Tetralogy of Fallot: A Systematic Review and Evidence Quality Assessment
Tetralogy of Fallot (ToF) is one of the most common cyanotic congenital heart defects. We sought to summarize all available data regarding the epidemiology and perioperative outcomes of syndromic ToF patients. A PRISMA-compliant systematic literature review of PubMed and Cochrane Library was performed. Twelve original studies were included. The incidence of syndromic ToF was 15.3% (nβ=β549/3597). The most prevalent genetic syndromes were 22q11.2 deletion (47.8%; 95% CI 43.4β52.2) and trisomy 21 (41.9%; 95% CI 37.7β46.3). Complete surgical repair was performed in 75.2% of the patients (nβ=β161/214; 95% CI 69.0β80.1) and staged repair in 24.8% (nβ=β53/214; 95 CI 19.4β30.9). Relief of RVOT obstruction was performed with transannular patch in 64.7% (nβ=β79/122; 95% CI 55.9β72.7) of the patients, pulmonary valve-sparing technique in 17.2% (nβ=β21/122; 95% CI 11.5β24.9), and RV-PA conduit in 18.0% (nβ=β22/122; 95% CI 12.1β25.9). Pleural effusions were the most common postoperative complications (nβ=β28/549; 5.1%; 95% CI 3.5β7.3). Reoperations were performed in 4.4% (nβ=β24/549; 95% CI 2.9β6.4) of the patients. All-cause mortality rate was 9.8% (nβ=β51/521; 95% CI 7.5β12.7). Genetic syndromes are seen in approximately 15% of ToF patients. Long-term survival exceeds 90%, suggesting that surgical management should be dictated by anatomy regardless of genetics