Prognia
Back to Guidelines
Society for Cardiovascular Angiography and InterventionsCardiology2020advanced

Multimodality Assessment of Congenital Coronary Anomalies

Published by American Society of Echocardiography

191References
3Tables
36Figures

Summary

AI-generated

Congenital coronary artery anomalies are recognized as important lesions with significant potential morbidity and mortality, including sudden cardiac death. Multimodality imaging techniques have replaced cardiac catheterization as the primary tool for characterization of most congenital CA anomalies in children.

congenital coronary anomaliesAAOCAALCAPATTECCTCMRASEcardiology

Key Takeaways

  • 1
    Echocardiography (TTE) is the initial screening and diagnostic tool of choice for suspected congenital coronary anomalies.
  • 2
    Cardiac computed tomography (CCT) and cardiac magnetic resonance (CMR) are essential complementary techniques for confirming complex anatomy, assessing intramural courses, and defining spatial relationships.
  • 3
    Delineating the exact origin and course of the anomalous coronary is critical for surgical planning and risk stratification in isolated CA anomalies like AAOCA and ALCAPA.
  • 4
    In complex congenital heart diseases (e.g., TOF, truncus arteriosus), precise preoperative delineation of a coronary artery crossing the right ventricular outflow tract (RVOT) is vital to prevent surgical injury.
  • 5
    Coronary angiography remains critical prior to right ventricular decompression in PA-IVS to evaluate for RV-dependent coronary circulation.

Key Recommendations

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

Scope & Objectives

Clinical Topic

Congenital Coronary Anomalies

Objectives

To provide multimodality guidelines for optimization of imaging for congenital coronary anomalies, with a review of the benefits and limitations of different imaging techniques.

Target Patient Population

Children and adults with suspected or confirmed congenital coronary anomalies

Target Providers

EchocardiographersCardiologistsPediatric CardiologistsRadiologistsSonographers

Patient Criteria & Setting

Therapeutic Area

Cardiovascular Disease

Guideline Scope

Imaging GuidelinesDiagnosisClinical Assessment

Care Settings

Outpatient ClinicsHospitalsImaging Centers

Special Populations

PediatricsNeonatesYoung Athletes

Safety & Contraindications

Contraindications

  • Implanted pacemakers or cardioverter defibrillators (relative contraindication for CMR)
  • Advanced renal disease (contraindication for gadolinium-based contrast in CMR)

Monitoring Guidance

Serial TTE is recommended for monitoring coronary ectasia, ventricular function, and regional wall motion abnormalities post-intervention or in conditions like anomalous left coronary artery from the pulmonary artery (ALCAPA).

Authors & Contributors

Peter FrommeltLeo LopezV. Vivian DimasBenjamin EidemB. Kelly HanH. Helen KoRichard LorberMasaki NiiBeth PrintzShubhika SrivastavaAnne Marie ValenteMeryl S. Cohen

Guideline Features

Multidisciplinary

Learning Context

Difficulty

advanced

Exam Relevance

Cardiology Board ExamsEchocardiography Board ExamsPediatric Cardiology Board Exams

Learning Paths

EchocardiographyCongenital Heart DiseaseCoronary Artery AnomaliesMultimodality ImagingCardiac Magnetic ResonanceCardiac Computed TomographyPediatric Cardiology