Scimitar syndrome: A new multipatch technique and incidence of postoperative pulmonary vein obstruction

Abstract

Objective: A review of our center's experience before March 2011 showed that one half of 36 patients who had a baffling or reimplantation procedure to repair scimitar syndrome developed pulmonary vein obstruction. We analyzed the results of a new operation that enlarges the left atrium and avoids circuitous pathways or tension on the scimitar pulmonary vein.Methods: Between April 2011 and November 2018, 22 patients underwent scimitar vein surgery; 11 had baffling or reimplantation and 11 only had the new operation that included resection of the atrial septum with removal of the muscular limbus. The left atrium was pulled down toward the scimitar vein and a V-shaped incision made at the scimitar vein atrial junction with the space filled with a pulmonary homograft. If the scimitar vein coursed adjacent to the atrium, a V-shaped incision was made into the scimitar vein and directly anastomosed to the atrium. A patch of autologous pericardium was used to septate the atrium and an additional patch placed anteriorly to augment the inferior vena cava.Results: Of the 11 patients who had baffling or reimplantation, 5 developed pulmonary vein obstruction between 45 days and 9.5 months after surgery associated with baffle thrombosis or tension on the pulmonary vein. None of the 11 patients who only had the new procedure developed pulmonary vein obstruction during postoperative monitoring up to 3.6 years.Conclusions: Patients having only the multipatch procedure for repair of scimitar syndrome have not developed postoperative pulmonary vein obstruction in the short to intermediate term. (JTCVS Techniques 2020;4:208-16) New surgical technique enlarges left atrium and avoids kinking or tension on pathway.

Commentary: Novel repair technique for scimitar syndrome

“…The described multipatch repair was efficacious and safe in the short- and medium term and it had significantly lower rates of pulmonary vein obstruction compared with reimplantation and intracardiac baffle techniques. 1 The results delineated by Geggel and colleagues 1 are very encouraging. We look forward to the authors providing future long-term follow-up regarding the development of pulmonary vein obstruction related to the multiple patches and the development of arrhythmias.…”

“…Geggel and colleagues 1 describe a multipatch repair technique for scimitar syndrome. This retrospective cohort study delineates excellent short- and midterm results.…”

“…The authors postulate that their multipatch repair enlarges the left atrium; avoids acute angles within the baffle pathway; and allows for durable, unobstructed pulmonary venous return to the left atrium. 1 …”

“…In the series by Geggel and colleagues, 1 22 patients underwent scimitar vein repair of which the novel, multipatch repair was used in half of patients (n = 11). The multipatch repair involves complete resection of the atrial septum (including the superior muscular limbus), shifting of the posterior left atrial wall rightward, maintenance of the scimitar vein connection to the IVC, and patch augmentation of the systemic venous return to the right atrium.…”

“…Geggel and colleagues 1 present a novel repair technique to management of scimitar syndrome. The described multipatch repair was efficacious and safe in the short- and medium term and it had significantly lower rates of pulmonary vein obstruction compared with reimplantation and intracardiac baffle techniques.…”