Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia

Abstract

A 6-month-old boy was diagnosed with coronary sinus orifice atresia, double-outlet right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and moderate common atrioventricular valve regurgitation associated with heterotaxy syndrome. Cardiac venous flow drained through a persistent left superior vena cava. We decided to perform coronary sinus orifice unroofing through the right atrium under a guide using a bougie. The persistent left superior vena cava was divided. Bidirectional Glenn anastomosis and edge-to-edge common atrioventricular valve repair were concomitantly performed. After a 1-year follow-up period, the patient is alive and well without any ischemic event.

Coronary sinus ostial atresia with persistent left superior vena cava demonstrated on cardiac CT in an infant with a functional single ventricle

“…As in our patient, CSOA and PLSVC without coronary sinus unroofing should be recognized preoperatively in patients with a functional single ventricle to avoid fatal coronary venous hypertension. As advocated by other reports [6,7], coronary sinus unroofing was performed before bidirectional cavopulmonary connection in our patient.…”

Transcatheter treatment of acquired coronary sinus ostium atresia in a child with complex congenital heart disease

“…In addition, lack of recognition of this condition can lead to operative fatalities if the decompressing left SVC is cannulated for cardiopulmonary bypass or ligated . There have been several reported cases of surgical unroofing of the CS at the time of stage 2 or 3 surgical palliation for this population, but only two prior reports of a transcatheter approach . The value of this approach is the potential to avoid additional surgeries and the associated risks while still improving the physiology.…”

“…However, there have been reports of symptomatic individuals with obstructed cardiac venous return secondary to CSOA without alternate/collateral drainage. Some of the cases were identified postmortem, while others were successfully treated by surgical and transcatheter approaches . Importantly, in children with single ventricle congenital heart disease who undergo staged surgical palliation, ligation of a persistent left SVC that drains the coronary sinus (CS) can be fatal .…”

Light-guided surgery to repair coronary sinus orifice atresia with left superior vena cava

“…Takabayashi and associates introduced a bougie as large as 2.5 mm in diameter into the coronary sinus. Nevertheless, they found it difficult to determine the location of the first left atrial incision [8]. The use of an oversized probe or overly forceful palpation may injure the coronary sinus.…”

“…Coronary sinus hypertension caused by LSVC ligation or cavopulmonary connection may induce serious consequences [1][2][3][4][5]. The surgical creation of unobstructed coronary sinus drainage, that is to say, coronary sinus redirection, is advised in patients undergoing LSVC ligation or cavopulmonary connection [5][6][7][8].…”