Double-Outlet Right Ventricle Fallot Type versus Tetralogy of Fallot: Comparison of Native Anatomy, Surgical Patterns, and Outcomes

Background: The conotruncal anomalies double-outlet right ventricle Fallot type (DORV TOF) and tetralogy of Fallot (TOF) show similar characteristics such as hemodynamics and repair techniques. However, estimates of survival and morbidity of this morphological TOF subgroup are currently scarce. We sought to describe native anatomy, surgical patterns and outcomes in patients with DORV TOF in comparison to classic TOF.

Method: All consecutive DORV TOF and TOF patients who underwent surgical repair at our institution from January 2004 to March 2022 were identified. Patients’ age and weight, size of pulmonary valve and pulmonary artery branches, surgical strategies, perioperative details, survival, and any reintervention rates at 5 and 10 years were analyzed.

Results: We identified 282 subjects including 52 DORV TOF (18%) and 230 TOF patients (82%). Mean follow-up times were 7.2 + 5.0 years. DORV TOF patients underwent more staging procedures (DORV TOF: 52% vs. TOF: 20%) and had lower preoperative peripheral oxygen saturation levels (DORV TOF: 84% vs. TOF: 90%). There was no difference according to mean age (7 ± 13 months vs. 7 ± 5 months), mean weight (7 ± 2 kg vs. 7 ± 5 kg), mean size of pulmonary valve annulus (8 ± 2 mm vs. 8 ± 2 mm) and right (6 ± 1 mm vs. 6 + 2 mm) and left pulmonary artery (5 ± 2 vs. 5+2 mm) between DORV TOF and TOF patients at repair. Conduits were more often used to achieve RVPA continuity in the DORV TOF group (DORV TOF: 35% vs. TOF: 1%). Infants with DORV TOF had longer extracorporeal cardiopulmonary bypass times (DORV TOF: 132 + 66 minutes vs. TOF: 103 + 33 minutes) and stayed longer on CICU (DORV TOF: 9 + 11 days vs. TOF: 5 + 3 days) and in the hospital (DORV TOF: 24 + 21 days vs. TOF: 16 + 20% days). Survival was similar in both groups at 5 and 10 years (5 years: DORV TOF: 96 + 3% vs. TOF 98 + 1%; 10 years: DORV TOF: 96 + 3% vs. TOF 98 + 1%). DORV TOF patients showed a higher rate of any reintervention at 5 and 10 years (5 years: DORV TOF: 62 ± 7% vs. TOF 86 ± 3%; 10 years: DORV TOF: 48 + 8% vs. TOF 79 + 3%).

Conclusion: The anatomical type of TOF influences surgical patterns, postoperative course, and outcomes. While midterm survival was similar between DORV TOF and TOF patients, reinterventions were more often required. Our analysis provides estimates of key markers of prognosis for DORV TOF patients that may be used to improve risk stratification and enrich family counseling.

Publication History

Article published online:
28 January 2023