Objective: The study aimed to evaluate the outcome of transatrial-transpulmonary repair of tetralogy of Fallot in relation to a right-ventricular outflow tract (RVOT)-sparing surgery.
Methods: Based on the surgical management of right-ventricular outflow tract obstruction (RVOTO) at repair of tetralogy of Fallot, 140 children were retrospectively divided into three groups: (1) pulmonary valve (PV)-sparing, (2) infundibulum-sparing and (3) extended trans-annular patch (TAP). Clinical and echocardiographic outcome was assessed with regards to three equally divided study time eras between January 1994 and June 2010.
Results: Over a 15-year study period, median age decreased from 11 (2-101) to 5 (1-11) months (p<0.001), whereas type of RVOT repair changed significantly between the first and the last era (group 1: 18-40%, group 2: 25-40% vs group 3: 57-20% (p=0.002)). Mortality was 0%. Complications were mainly related to clinical restrictive RV physiology (27%) and arrhythmia (10%). This cardiac morbidity remained constant over the eras and was associated with younger age (p=0.04), increased postoperative right ventricle/left ventricle (RV/LV) pressure ratio (p=0.01) and type of RVOT repair at the cost of TAP (p=0.03). Median follow-up of 8 years (1-16 years) showed an overall freedom from RVOT re-operation of 84% and 73%, respectively at 5 and 10 years. Most re-operations were for residual/recurrent RVOTO (12%) occurring more frequently in the latter era: 16% versus 7% in era 1 (p=0.08). Late echocardiographic evaluation revealed a strong correlation between severity of pulmonary regurgitation and increased RV/LV size ratio, which was mainly determined by increased TAP length (p<0.001) and duration of follow-up (p=0.06).
Conclusion: In a 15-year's experience with transatrial-transpulmonary correction of tetralogy of Fallot, a valve- and infundibulum-sparing approach has been advanced by lowering the age for elective repair. This change has been performed without compromising immediate clinical outcome, despite an increased early re-operation rate for residual obstruction. However, longer follow-up will disclose whether this approach is protective against progressive and late RV dysfunction.