Clinical Features and Surgical Outcomes of Coronary Sinus Orifice Atresia

Abstract

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

A Rare Associated Anomaly That Should Be Remembered In Patients Undergoing Univentricular Palliation: Coronary Sinus Ostial Atrezia

“…Surgical ligation of the left persistent superior vena may cause death in undiagnosed patients ( 3 , 4 , 6 ). It has been previously described that surgical procedures (redirection or unroofing) can be performed to prevent complications after Glenn and/or Fontan operations ( 1 , 3 ). In our patient, pulmonary artery growth was considered to be inadequate for successful Fontan operation, and medical follow-up with later Fontan completion along with coronary sinus unroofing was planned.…”

“…Coronary sinus ostium atresia associated with congenital heart diseases with single ventricular physiology is rare and detection of this anomaly is very important ( 1 - 3 ). In coronary sinus ostial atresia, the coronary sinus usually drains into an unroofed coronary sinus and the left atrium.…”

Single-Stage Fontan Operation in a Patient With Coronary Sinus Ostium Atresia

“…In single ventricle cases, the clinical picture may be complicated. [1][2][3] In this report, we present a case of CSOA that was surgically repaired during the Fontan operation.…”

“…Ostial atresia of the coronary sinus is a rare congenital anomaly in which the coronary sinus is usually unroofed and drains into the left atrium (LA). 1,2 Patients with coronary sinus ostial atresia (CSOA) usually do not require surgical intervention, especially if the coronary sinus is naturally unroofed into the LA or drains via a left superior vena cava (LSVC). In single ventricle cases, the clinical picture may be complicated.…”

Angiographically detectable Thebesian veins are a dynamic and reversible finding in the setting of congenital heart disease

“…Prominent ThVs could allow for an alternative route of myocardial venous drainage, and it has been established that ThVs are capable of carrying the bulk of venous return in situations where the epicardial coronary veins are compromised, or in the setting of coronary sinus ostial atresia. [5][6][7] Some investigators hypothesize that ThVs provide an alternative channel of nutrition to the myocytes in the setting of proximal coronary artery obstruction (via retrograde flow from the ventricles to the capillary network). Alternatively, high ThV burden (particularly the arterioluminal variety) can potentially be detrimental, hypothesized to cause coronary steal and myocardial ischemia in patients without coronary lesions or obstructions.…”