Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes

Background: This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot on pulmonary valve annulus growth, the rate of valve-sparing repair at the time of intracardiac repair, and long-term functional outcomes. Methods: This retrospective study involved 330 patients with tetralogy of Fallot who underwent intracardiac repair between 1991 and 2019 and included 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0 ± 7.3 years. We compared the data of patients who underwent BTS and patients who did not undergo BTS before intracardiac repair. Results: The median age before BTS was 71 days (range, 28 to 199) and the median body weight was 4.3 kg (range, 3.3 to 6.8 kg). There were no inhospital or interstage deaths after BTS. The pulmonary valve annulus Z scores of patients with BTS revealed significant growth after BTS (from −4.2 ± 1.8 to −3.0 ± 1.7, P < .001). Valve-sparing repair was eventually performed in 207 patients (63%), including 26 (46%) who underwent staged repair. The overall freedom from pulmonary regurgitation-related reintervention was 99.7%, 99.1%, and 95.8% at 1, 5, and 20 years, respectively. Conclusions: A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. Blalock-Taussig shunt may have contributed to the avoidance of primary transannular patch repair and facilitated pulmonary valve annulus growth; therefore, approximately half of the symptomatic neonates and infants were recruited for valve-sparing repair. Staged repair may have led to functionally reliable delayed transannular patch repair, thereby resulting in fewer surgical reinterventions.