Double Outlet Right Ventricle—Follow-up and Outcome after Surgical Repair

Objectives: The objective of this study was to report the outcomes of biventricular repair in patients with true double outlet right ventricle (DORV) and to determine risk factors for early or late mortality and reoperations.

Methods: True DORV was diagnosed in 236 patients in our institution between October 1977 and August 2016. Biventricular repair was achieved in 147 patients. Univentricular palliated patients (n = 62) were not included in this retrospective study. Further 27 patients were not eligible to repair after first-stage palliation. Results were compared among three groups depending on the location of the ventricular septal defect (VSD) and concomitant anatomic malformations.

Results: Patients with DORV and VSD in subaortic location (group 1, mean age at repair 30.1 months) were considered separately according to the presence (n = 73) or absence (n = 21) of pulmonary stenosis. Group 2 included patients with subpulmonary VSD (n = 19, mean age at repair 8.2 months) who underwent arterial switch operation in 68.4%. In the third group (complex DORV, n = 34), different “customized” repairs were performed. A previous palliation was implemented in 39.5%: banding of the pulmonary artery in 13 patients (12.4%), central aortopulmonary shunt in 18 (31%), and modified Blalock–Taussig shunt in four patients (6.9%). The survival rate after 20 years was highest in group 1 with 88.8% (95% confidence interval [CI]: [78.2–99.4]), 73.3% in group 2 (95% CI: [53.3–93.3]), and 71.7% in group 3 (95% CI: [40.7–100]). Reoperation-free survival after 10 years was highest in group 1 with 73.0% (95% CI: [62.2–83.8]) and group 3 with 50.9% (95% CI: [21.5–80.3]). Reinterventions were frequent in patients with subpulmonary VSD (n = 9, 47.3%) and were often caused by left ventricular outflow tract obstruction. Right-ventricle-to-pulmonary-artery (RVPA) conduits (p < 0.0001) and right-ventricular-to-outflow-tract (RVOT) patching (p = 0.018) were identified as significant risk factors for reoperation in multivariate analysis. Follow-up was 91.2% complete with a mean follow-up time of 10.8 years after repair (95% CI: [9.3–12.3]).

Conclusion: Patients with subpulmonary VSD had the most unfavorable outcome 10 years after repair regarding mortality and reoperation rates. Favorable outcomes were achieved in all other DORV subtypes. Not surprisingly, RVPA conduits and RVOT patches were identified as main risk factors for later reoperation.