Management of Dilated Coronary Sinus with ASD and Anomalous Pulmonary Venous Drainage
Diagnostic Clarification and Advanced Imaging
- The American College of Cardiology recommends CMR or cardiac CT to precisely map all pulmonary venous connections and define the exact anatomy of the coronary sinus and atrial septal defect 1, 2
- CMR is superior for identifying anomalous pulmonary venous connections that may be difficult or impossible to visualize by echocardiography, particularly those connecting to the coronary sinus 3
- TEE should be performed to visualize the entire atrial septum, assess for unroofing of the coronary sinus, and identify any persistent left superior vena cava (PLSVC) that drains into the coronary sinus 3, 4
Hemodynamic Assessment
- Cardiac catheterization can be useful to define precise hemodynamics including shunt quantification (Qp:Qs ratio), pulmonary artery pressures, and pulmonary vascular resistance 1, 2
- Pulse oximetry at rest and with exercise should be performed to identify shunt direction and detect any right-to-left shunting that would contraindicate closure 3
Surgical Indications and Timing
- The American College of Cardiology recommends surgical repair when there is right ventricular volume overload with a hemodynamically significant shunt (Qp:Qs ≥1.5:1), provided pulmonary artery systolic pressure is less than 50% of systemic pressure and pulmonary vascular resistance is less than one-third of systemic resistance 1, 2
- Repair of the anomalous pulmonary venous connection is mandatory at the time of ASD closure 1, 2
Contraindications to Closure
- The American College of Cardiology recommends that ASD closure should NOT be performed when PA systolic pressure is greater than two-thirds of systemic pressure, pulmonary vascular resistance is greater than two-thirds of systemic resistance, or there is a net right-to-left shunt (Eisenmenger physiology) 1, 2