Anatomical Correction for Left Superior Vena Cava Related Obstruction—A “Novel” Surgical Technique Using Just Native Tissue

Objective: Left superior vena cava (LSVC)-related obstruction of cardiac structures is a rare finding in patients with complex cardiac anomalies. We report our experience by establishing a left superior to right superior caval vein continuity (“innominate vein creation by direct LSVC–RSVC end-to-side anastomosis”) and coronary sinus unroofing if indicated for LSVC-related intracardiac or extracardiac obstructions.

Methods: Seventeen patients (median age: 0.9 years; range: 7 days–40 years) underwent anatomical correction of LSVC without the use of foreign material in conjunction with repair or palliation of congenital anomalies in a single center between April 2015 and March 2018. Indications for the procedure were LSVC-related obstruction of left superior pulmonary vein (n = 1), left pulmonary artery (n = 5), and left ventricular inflow due to a dilated coronary sinus (n = 11). Additional procedures included mitral (n = 3) or atrioventricular (n = 2) valve surgery, right ventricular to pulmonary artery conduit (n = 3), first stage palliation (n = 2), or biventricular repair (n = 3) of hypoplastic left heart complex, sutureless repair left pulmonary vein occlusion (n = 1), and repair of absent pulmonary valve syndrome (n = 1).

Results: All LSVC or coronary sinus-related obstructions were effectively relieved. One patient died early due to low cardiac output not related to the venous procedure. One patient needed stenting of the superior vena cava below the unobstructed cephalad vein anastomosis at the former RSVC cannulation site. Follow-up was complete and presented a 94.1% survival after 2 years. Innominate vein patency was 100% documented by echocardiography (n = 15) or cardiac catheterization (n = 6).

Conclusion: Anatomical correction by surgical creation of an innominate vein is an effective method to relieve LSVC-related obstructions. Further follow-up studies including quantification of cardiac Z-scores growth potential are needed to evaluate the impact of coronary sinus unroofing.