Restrictive LV-PA conduit in ccTGA with VSD and LVOTO

Abstract

Introduction

Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO.

Objective

To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure.

Methods

Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children's Hospitals (Paris, France).

Results

Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10 years. Late reintervention consisted in LV-PA conduit replacement (n = 3), PA stenting (n = 1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, n = 1). At last follow-up, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation.

Conclusion

We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical.