Multimodality Assessment of Congenital Coronary Anomalies

Publication Date: March 1, 2020
Last Updated: March 14, 2022

Key Points

Recommendations

Anomalous Aortic Origin of a Coronary Artery

  • AAOCA is relatively common and usually benign, but certain forms, particularly when a CA courses between the great arteries, are associated with sudden death.
  • Echocardiography identifies AAOCA with an interarterial course, and important markers include:
    • Oblique origin of the anomalous CA from the opposite sinus
    • When intramural, an anomalous course within the anterior aortic wall with an acute angled origin that can be demonstrated by color Doppler mapping
    • When intraconal, an anomalous course within the muscular conal septum inferior to the pulmonary valve annulus
  • Complementary imaging techniques, particularly CCT and CMR, can help define AAOCA anatomy, specifically the ostia, caliber, and distal branching, but accurate risk stratification for sudden death remains elusive.
  • The utility of myocardial stress imaging and IVUS in asymptomatic patients in decision-making about intervention is also unclear.
6731

Recommended Imaging Strategy for Anomalous Aortic Origin of a Coronary Artery

  • TTE should be the initial screening tool for suspected AAOCA, particularly in forms that are associated with myocardial ischemia (those with an interarterial course).
  • When an interarterial AAOCA is identified, delineation of anomalous ostial origin/size and course should be confirmed with CCT and/or CMR.
  • Resting and stress myocardial perfusion assessment may be used to help identify ischemia and/or correlate symptoms in AAOCA.
6731

Anomalous Left Coronary Artery from the Pulmonary Artery

  • ALCAPA is a rare but treatable cause of dilated cardiomyopathy in infants and children and should be ruled out at presentation.
  • Echocardiography is the diagnostic imaging modality of choice in ALCAPA, but other imaging techniques may be necessary if the diagnosis is uncertain.
  • Characteristic echocardiographic hallmarks in the diagnosis of ALCAPA include:
    • Direct visualization of the origin of the LCA from the PA by 2D imaging
    • Retrograde color flow Doppler from the LCA into the PA
    • Extensive RCA dilation and tortuosity by 2D imaging
    • Prominent intramyocardial color Doppler signals within ventricular septum and LV myocardium due to dilated RCA collaterals
    • Significant LV chamber dilation and dysfunction
    • Mitral valve papillary muscle fibrosis with significant MR
6731

Recommended Imaging Strategy for Anomalous Left Coronary Artery from the Pulmonary Artery

  • TTE should be the primary screening tool and is usually diagnostic in suspected ALCAPA in infants and children.
  • 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.
  • After repair, CCT and/or CMR may be used when there are concerns about coronary anatomy and/or persistent myocardial perfusion abnormalities.
6731

Key Points for Isolated Coronary Artery Fistulas

  • CAFs are rare and frequently benign, but fistulas can enlarge over time and lead to myocardial ischemia and/or heart failure.
  • Echocardiography may identify CAFs, with common markers including:
    • Dilation of the involved CA with increased flow by color Doppler
    • Turbulent continuous flow throughout the length of the involved CA when draining into a low-pressure chamber (usually the right heart)
    • Turbulent flow into the chamber at the exit point of the CAF by color Doppler
  • Alternative imaging techniques (CCT, CMR, angiography) can clearly identify the CAF anatomy/hemodynamics and should be employed when intervention is considered.
6731

Recommended Imaging Strategy for Isolated Coronary Artery Fistulas

  • TTE should be the initial screening tool for suspected CAF and is effective in serial monitoring of size/physiologic effects.
  • When intervention for a significant CAF is planned, delineation of origin, course and exit should be confirmed with cardiac catheterization, CCT or CMR.
  • Supplementary hemodynamic information may be obtained by cardiac catheterization or CMR when the need for closure is unclear.
  • Coronary ectasia following CAF closure can persist and should be monitored for development of thrombus/ischemia.
6731

Congenital Coronary Anomalies Associated with Supravalvular Aortic Stenosis

  • SVAS can be associated with potentially life-threatening CA stenosis.
  • Echocardiography is limited in diagnosing CA stenosis in SVAS, but identification of an ostial location near the sinotubular ridge increases risk of stenosis.
  • Alternative imaging techniques (CCT, CMR, angiography) can help identify CA stenosis, but use of deep sedation during these procedures can trigger an ischemic event.
6731

Recommended Imaging Strategy for Congenital Coronary Anomalies Associated with Supravalvular Aortic Stenosis

  • TTE may be an effective screening tool for CA anomalies with SVAS, particularly in identification of anatomic subtypes associated with ischemia.
  • CCT is the preferred imaging modality to document/confirm coronary ostial abnormalities, since it can be performed without deep sedation/anesthesia in this high-risk population.
6731

Congenital Coronary Anomalies Associated with Transposition of the Great Arteries

  • Variations in CA origin and course are common in TGA and can impact surgical planning and risk of postoperative CA complications.
  • Echocardiography may identify concerning CA patterns in TGA preoperatively, particularly an intramural course between the great arteries and CAs coursing anterior to the aorta or posterior to the pulmonary artery.
  • Ventricular dysfunction and regional wall motion abnormalities after the arterial switch operation should prompt alternative imaging techniques (CCT, CMR, angiography) to assess CA patency.
6731

Recommended Imaging Strategy for Congenital Coronary Anomalies Associated with Transposition of the Great Arteries

  • TTE should be the primary imaging tool for delineation of coronary artery anatomy in the preoperative assessment of TGA.
  • When an intramural coronary is suspected, clear delineation of this higher-risk variant using CCT may be warranted prior to arterial switch surgery.
  • Postoperative CCT, CMR, and/or cardiac catheterization should be performed when there is suspicion of coronary compromise.
6731

Congenital Coronary Anomalies Associated with Tetralogy of Fallot

  • Variations in CA origin and course are common in TOF and can impact initial surgical planning and risk of CA complications during later interventions.
  • Echocardiography identifies CA branches from the RCA crossing the RVOT prior to initial surgical repair.
  • Alternative imaging techniques (CCT, CMR, angiography) assessing coronary artery relationships to the RVOTare necessary prior to pulmonary valve replacement.
6731

Recommended Imaging Strategy for Congenital Coronary Anomalies Associated with Tetralogy of Fallot

  • TTE should be the primary screening tool for delineation of coronary anatomy in the preoperative assessment of TOF.
  • After primary repair, delineation of coronary anatomy by CCT or CMR should be performed prior to pulmonary valve replacement.
  • In patients undergoing transcatheter pulmonary valve replacement, coronary angiography may be sufficient to assess coronary anatomy during the procedure.
6731

Congenital Coronary Anomalies Associated with Truncus Arteriosus

  • Variations in CA origin and course are common in truncus arteriosus, including single CA, and can impact risk of CA complications during surgical interventions.
  • Echocardiography may identify CA origins and the location/ proximity of the CA ostia to the pulmonary arteries prior to initial surgical repair.
  • Alternative imaging techniques (CCT, CMR, angiography) are necessary to assess CA course prior to subsequent pulmonary valve replacements.
6731

Recommended Imaging Strategy for Congenital Coronary Anomalies Associated with Truncus Arteriosus

  • TTE should be the primary screening tool for delineation of coronary anatomy in the preoperative assessment of truncus arteriosus.
  • After primary repair, delineation of coronary anatomy by CCT or CMR should be performed prior to pulmonary valve replacement.
  • In patients undergoing transcatheter pulmonary valve replacement, coronary angiography may be sufficient to assess coronary anatomy during the procedure.
6731

Congenital Coronary Anomalies Associated with Pulmonary Atresia with Intact Ventricular Septum

  • Delineation of RV-dependent CA circulation is critical prior to RV decompression.
  • Identification of abnormal RV-CA connections can be made with echocardiography, but confirmation of the anatomy is required prior to intervention and best accomplished by angiography.
  • Tricuspid valve Z-score < 2.5 is a risk factor for poor prognosis with biventricular repair and for the presence of abnormal RV-CA connections.
  • CMR is a useful tool to evaluate for myocardial abnormalities related to abnormal coronary perfusion both in the short and long term.
6731

Recommended Imaging Strategy for Congenital Coronary Anomalies Associated with Pulmonary Atresia with Intact Ventricular Septum

  • TTE may identify anatomic/physiologic features concerning for coronary anomalies, but it is usually inadequate as the sole tool to assess coronary anatomy in PA/IVS.
  • Coronary angiography should be performed prior to any interventions that would result in decompression of the RV.
6731

Congenital Coronary Anomalies Associated with Hypoplastic Left Heart Syndrome

  • CA abnormalities are more common in the HLHS subtype of MS/AA.
  • Echocardiography is typically adequate for the assessment of abnormal CA communications in patients with HLHS.
  • Patients with abnormal CA communications are palliated in a similar manner to other subtypes of HLHS with outcomes that are largely similar.
  • Direct imaging with angiography is typically reserved for postoperative patients in whom there is evidence of ischemia.
6731

Recommended Imaging Strategy for Congenital Coronary Anomalies Associated with Hypoplastic Left Heart Syndrome

  • TTE should be the primary screening tool for delineation of coronary anatomy in the preoperative assessment of HLHS.
  • Coronary angiography may be performed in the rare circumstance where abnormal LV-CA connections are suspected.
6731

Recommendation Grading

Abbreviations

  • 2D: 2-dimensional
  • AAOCA: Anomalous Aortic Origin Of A Coronary Artery
  • AAOLCA: Anomalous Aortic Origin Of The Left Coronary Artery
  • AAORCA: Anomalous Aortic Origin Of The Right Coronary Artery
  • ALCAPA: Anomalous Left Coronary Artery From The Pulmonary Artery
  • ARCAPA: Anomalous Right Coronary Artery From The Pulmonary Artery
  • CA: Coronary Artery
  • CAF: Coronary Artery Fistula
  • CCT: Cardiac Computed Tomography
  • CHD: Congenital Heart Disease
  • CMR: Cardiac Magnetic Resonance
  • ECG: Electrocardiography
  • HLHS: Hypoplastic Left Heart Syndrome
  • LAD: Left Anterior Descending
  • LCA: Left Coronary Artery
  • LCx: Left Circumflex
  • LV: Left Ventricle
  • MR: Mitral Regurgitation
  • PA: Pulmonary Artery
  • PET: Positron Emission Tomography
  • RCA: Right Coronary Artery
  • RV: Right Ventricle
  • SPECT: Single-photon Emission Computerized Tomography
  • SVAS: Supravalvular Aortic Stenosis
  • TEE: Transesophageal Echocardiography
  • TGA: Transposition Of The Great Arteries
  • TOF: Tetralogy Of Fallot
  • TTE: Transthoracic Echocardiography
  • VSD: Ventricular Septal Defect

Overview

Title

Multimodality Assessment of Congenital Coronary Anomalies

Authoring Organizations

Publication Month/Year

March 1, 2020

Last Updated Month/Year

February 5, 2024

Supplemental Implementation Tools

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Inclusion Criteria

Female, Male, Adolescent, Adult, Child, Older adult

Health Care Settings

Emergency care, Hospital, Operating and recovery room, Outpatient, Radiology services

Intended Users

Radiology technologist, nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Diagnosis, Prevention

Diseases/Conditions (MeSH)

D003324 - Coronary Artery Disease, D004452 - Echocardiography, D006330 - Heart Defects, Congenital

Keywords

congenital heart disease, echocardiography, Coronary artery, Congenital heart disease