Unusual Presentation of Unroofed Coronary Sinus With Cyanosis After Ventricular Septal Defect Closure

Abstract

Cyanosis after surgical closure of ventricular and/or atrial septal defect is very unusual. We report a case of unroofed coronary sinus in a six-month-old boy who developed cyanosis after surgical closure of ventricular and atrial septal defects. Clinical presentation, diagnosis, and management are discussed.

CT angiography for diagnosis and subcategorization of unroofed coronary sinus syndrome

“…Another 4 surgical patients who had been preoperatively diagnosed with URCS by CTA, no information was available regarding the management of URCS in their previous operative reports. It's worth noting that URCS may be missed by imaging or even during surgical repair due to its hidden location (26)(27)(28). The chances of leaving URCS untreated during surgical operation was possibly even higher, which occurred in 7 out of 30 patients in this series.…”

Low Oxygen Saturation Following Total Correction in a Patient with Tetralogy of Fallot and Persistant Left Superior Caval Vein - How Did We Diagnose and Manage?

“…7 Yarrabolu et al reported that they found UCS in a 6-month-old boy who developed cyanosis after surgical closure of VSDs. 8 Mallula et al reported that they successfully completed the PLSVC and coronary sinus ASD in a 2-month-old case with TOF. 3 Ramman et al found UCS in a patient with operated TOF and PLSVC who developed post-operative hypoxia.…”

Unroofed Coronary Sinus Syndrome Presenting with Early Pulmonary Hypertension

“…The pulmonary resistance was also increased to 3.8 Woods units. The pulmonary pressure gradually decreased and the URCS was monitored clinically [3]. The pulmonary hypertension was initially managed with inhaled nitric oxide and supplemental oxygen and later with oral Sildenafil for 3 months.…”