In our Institute, one of 14 patients who underwent modified Fontan procedure due to various complex congenital anomalies between October 1985 and May 1990 was noted to have unroofed coronary sinus associated with left persistent superior vena cava (LPSVC) at surgery. This patient's pathology was double inlet left ventricle, transposition of great arteries, pulmonary stenosis and left persistent superior vena cava. LPSVC was known to drain into the CS. After the modified Fontan procedure, severe arterial hypoxia was noted. Cardiopulmonary bypass immediately recommenced. Unroofed CS was identified by retrograde-probing through the CS orifice. Leaving the CS orifice in the left atrium and Iigation of the LPSVC resulted in resolution of cyanosis. Postoperative period was uneventful. Unroofed CS may permit significant persistent right to left interatrial shunting after a modified Fontan procedure.
Keywords: Without sus- pended (unroofed) coronary sinus, modified Fontan procedure, persistent left superior vena cavaCopyright © 2025 Archives of the Turkish Society of Cardiology