ObjectiveChildren with congenital heart defects (CHD) requiring cardiovascular surgery (CVS) rarely require tracheostomy placement; however the mortality rate remains high. The study aimed to analyze the incidence of tracheostomy in children with CHD, and to determine factors contributing to postoperative outcomes, decannulation rates, and mortality.MethodsRetrospective case series of children ≤18 years old with CHD status post‐CVS who underwent tracheostomy placement between January 1, 2001 and December 31, 2020. Variables analyzed included demographic information, presence of comorbidities including prematurity, respiratory diseases, presence of genetic syndromes, decannulation status, type of repair (univentricular vs. biventricular), and need for cardiopulmonary bypass. Adverse events analyzed included all‐cause mortality, development of mediastinitis, fatal decannulation, and persistence of tracheocutaneous fistula.ResultsFifty‐one patients were analyzed. The incidence of tracheostomy was 0.8%. Median age at tracheostomy was 5.3 months. The 5‐year survival estimate was 56.3% (95% confidence interval 43.6%, 72.6%). Age ≤6 months at the time of tracheostomy placement (p = .03), and the presence of tracheomalacia (p = .04) were factors significantly associated with 5‐year survival. Two patients (3.9%) experienced fatal decannulation, and one patient (2.0%) developed postoperative mediastinitis. The 10‐year decannulation rate estimate was 47.8% (30.5%, 63.2%). Seven patients (13.7%) had a persistent tracheocutaneous fistula.ConclusionsThis study corroborates high mortality rates in this population. Factors associated with improved survival were younger age at the time of tracheostomy and presence of tracheomalacia. Decannulation rates were low, but estimates improved over 10 years. Further studies are needed to determine optimal indications and timing for tracheostomy placement in this patient population.Level of Evidence4