Multimodality Assessment of Congenital Coronary Anomalies

Anomalous Aortic Origin of a Coronary Artery

• REC-AAOCA-1

  TTE should be the initial screening tool for suspected AAOCA, particularly in forms that are associated with myocardial ischemia (those with an interarterial course).

  Diagnosis
• REC-AAOCA-2

  When an interarterial AAOCA is identified, delineation of anomalous ostial origin/size and course should be confirmed with CCT and/or CMR.

  Diagnosis

Anomalous Pulmonary Origin of a Coronary Artery

• REC-ALCAPA-1

  TTE should be the primary screening tool and is usually diagnostic in suspected ALCAPA in infants and children.

  Diagnosis
• REC-ALCAPA-2

  Confirmation of the diagnosis using CCT or CMR should be reserved for cases where the anatomy is unclear; cardiac catheterization is rarely needed for diagnosis.

  Diagnosis

Isolated Congenital Coronary Artery Fistulas

• REC-CAF-1

  When intervention for a significant CAF is planned, delineation of origin, course and exit should be confirmed with cardiac catheterization, CCT or CMR.

  Clinical Assessment

Transposition of the Great Arteries

• REC-TGA-1

  Postoperative CCT, CMR, and/or cardiac catheterization should be performed when there is suspicion of coronary compromise.

  Clinical Assessment

Pulmonary Atresia With Intact Ventricular Septum

• REC-PAIVS-1

  Coronary angiography should be performed prior to any interventions that would result in decompression of the RV.

  Clinical Assessment

Therapeutic Area

Guideline Scope

Care Settings

Special Populations

Comparable guidelines from other societies

• ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease (2008 and 2018)