Mid-term results of the Lugones procedure for scimitar syndrome repair

The study objective was to report the mid-term outcomes of the Lugones procedure (in situ pericardial rerouting technique) and its variants for scimitar syndrome repair.

We performed a multicenter retrospective descriptive study of patients undergoing scimitar syndrome repair with the Lugones procedure. Three variants were used: original, variant A with atrial septum repositioning, and variant B with an interatrial pericardial flap. Primary end points were survival, freedom from reoperation, and freedom from pulmonary venous obstruction. Follow-up was based on clinical assessment and serial echocardiography; computed tomography scan, magnetic resonance imaging/4-dimensional flow magnetic resonance imaging, or cardiac catheterization was obtained when echocardiographic assessment was limited, clinical or imaging findings were equivocal, or complementary anatomic/hemodynamic information was sought.

Thirty-eight patients from 11 institutions were included (median age, 5 years; range, 0.7-43). Preoperative scimitar vein stenosis was present in approximately 29%; aortopulmonary collaterals were embolized preoperatively in 24 patients (63%). The original procedure was performed in 28 cases, variant A in 5 cases, and variant B in 5 cases. In-hospital mortality was 0%. Early complications were uncommon (1 postoperative pulmonary venous pathway obstruction due to extrinsic compression; 1 transient diaphragmatic dysfunction). Mean follow-up was 74 months (maximum 13.5 years), with no late deaths, reoperations, recurrent obstruction, or additional late pulmonary venous pathway obstruction. Computed tomography scans consistently demonstrated wide reconstructed pathways; 4-dimensional flow magnetic resonance imaging showed physiological pulmonary venous flow patterns after repair.

The Lugones procedure yielded excellent mid-term outcomes with no deaths or reoperations and a very low incidence of postoperative pulmonary venous obstruction.