eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Anomalous Left Coronary Artery From the Pulmonary Artery: Differential Diagnoses & Workup

Author: Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Coauthor(s): Howard S Weber, MD, FAAP, FACC, FSCAI, Professor, Assistant Chief, Section of Pediatric Cardiology, Penn State University School of Medicine; Director, Pediatric Catheterization Laboratory, Milton S Hershey Medical Center
Contributor Information and Disclosures

Updated: Nov 7, 2008

Differential Diagnoses

Cardiomyopathy, Dilated
Coronary Artery Fistula
Mitral Valve Insufficiency
Myocarditis, Viral

Other Problems to Be Considered

Congenital mitral valve disorder
Idiopathic dilated cardiomyopathy

Workup

Laboratory Studies

  • Cardiac isoenzymes
    • Laboratory blood tests are not definitive in the diagnosis of anomalous left coronary artery arising from the pulmonary artery (ALCAPA).
    • Although elevation of creatine kinase (CK), MB, or troponin occurs following infarction of cardiac muscle, these tests should not be used for diagnostic purposes.

Imaging Studies

  • Chest radiography: This usually demonstrates cardiomegaly, with or without pulmonary venous congestion, although this is not diagnostic for ALCAPA.
  • Two-dimensional echocardiography with Doppler color flow mapping
    • This test often is diagnostic and, in some situations, replaces the need for cardiac catheterization and angiography.
    • Echo without Doppler may identify abnormal origin of the left coronary artery from the main pulmonary artery (Media file 3). In unusual circumstances, the anomalous coronary may arise from a branch pulmonary artery, making echocardiographic diagnosis difficult, even with Doppler.
  • The use of color flow velocity mapping can be diagnostic, demonstrating retrograde flow from the anomalous left coronary into the pulmonary trunk. The retrograde flow into the pulmonary trunk is typically directed in an unusual orientation within the main pulmonary artery (Media file 4), distinguishing it from the diagnosis of a patent ductus arteriosus.
  • Doppler mapping of an abnormal color flow jet will usually identify abnormal retrograde flow within the main pulmonary artery in both late systole and diastole (Media file 5). The mapping image partially depends on pulmonary artery pressure.
  • The presence of retrograde flow is dependent on the development of collaterals between the left and right coronary artery systems. If collateralization has not occurred, as may be the case with a very early age presentation, this finding may be absent.
  • Abnormal dilation of the proximal right coronary artery, when present, reflects development of extensive collateralization between the right and left coronary artery systems in those patients who present later in infancy or in childhood.
  • An additional finding, which is not sensitive but highly specific, is abnormal "brightness" (echogenicity) of left ventricular papillary muscles and sharply delimited sectors of the left ventricular endocardial surface.
  • Variable degrees of mitral valve regurgitation, left ventricular dysfunction, and wall motion abnormalities may be identified.4

Other Tests

  • Twelve-lead electrocardiography
    • Typically, an anterolateral infarct pattern with abnormal deep (>3 mm) and wide (>30 msec) q waves is observed in leads I, aVL, V5, and V6, absent q waves in leads II, III, and aVF, and poor R wave progression across the precordial leads, with sudden shift to qR. Electrocardiography (ECG) detects abnormalities of repolarization in the form of ST-segment depression or inversion, both inferior and lateral (Media file 1). The QRS axis is typically normal, although, in some cases, a left superior axis is seen.
    • Following successful surgical revascularization, the ECG may revert to normal findings with the disappearance of the pathologic q waves and ST-T wave changes (Media file 2).

Procedures

  • Cardiac catheterization and angiography
    • Angiographic evaluation of the coronary artery system should be performed despite a negative echocardiogram if either the clinical history or ECG is strongly suggestive.
    • Hemodynamic measurements are usually consistent with low cardiac output and elevated left atrial pressures secondary to reduced left ventricular compliance or significant mitral valve insufficiency.
    • Oximetry may show a small left-to-right shunt into the pulmonary arteries.
    • Aortography or selective right coronary arteriography usually demonstrates an enlarged right coronary artery system with collateralization to the left coronary artery and eventual reflux of contrast into the pulmonary arterial system (Media file 6).
    • If collateralization has not occurred, identification of the anomalous left coronary artery may not be evident by aortography or selective right coronary arteriography.
  • Stop flow angiography
    • With a large bolus of contrast under high pressure, an alternative approach is to perform a balloon occlusion angiogram within the distal main pulmonary artery, which retrogradely should fill the anomalous left coronary artery (Media file 7).
    • Though rare, false-negative results with this technique may be caused by incomplete occlusion of the main pulmonary artery or by balloon malposition. A balloon positioned in the proximal main pulmonary artery may occlude the orifice of the anomalous left coronary. Alternatively, if the anomalous left coronary artery arises from the left pulmonary artery, positioning the balloon in the distal main pulmonary artery may prevent contrast from entering the coronary artery.5

More on Anomalous Left Coronary Artery From the Pulmonary Artery

Overview: Anomalous Left Coronary Artery From the Pulmonary Artery
Differential Diagnoses & Workup: Anomalous Left Coronary Artery From the Pulmonary Artery
Treatment & Medication: Anomalous Left Coronary Artery From the Pulmonary Artery
Follow-up: Anomalous Left Coronary Artery From the Pulmonary Artery
Multimedia: Anomalous Left Coronary Artery From the Pulmonary Artery
References
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References

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  2. Fontana RS, Edwards JE. Congenital Cardiac Disease: a Review of 357 Case Studies Pathologically. WB Saunders; 1962:291.

  3. Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of anomalous left coronary artery from the pulmonary artery. Circulation. Aug 1980;62(2 Pt 2):I180-9. [Medline].

  4. Pisacane C, Pinto SC, De Gregorio P, et al. "Steal" collaterals: an echocardiographic diagnostic marker for anomalous origin of the left main coronary artery from the pulmonary artery in the adult. J Am Soc Echocardiogr. Jan 2006;19(1):107.e3-107.e6. [Medline].

  5. Piechaud JF, Shalaby L, Kachaner J, et al. Pulmonary artery "stop-flow" angiography to visualize the anomalous origin of the left coronary artery from the pulmonary artery in infants. Pediatr Cardiol. 1987;8(1):11-5. [Medline].

  6. McNamara DG. Treatment of anomalous origin of left coronary artery arising from the pulmonary artery. 1973;1:497-499.

  7. Meyer BW, Stefanik G, Stiles QR, et al. A method of definitive surgical treatment of anomalous origin of left coronary artery. A case report. J Thorac Cardiovasc Surg. Jul 1968;56(1):104-7. [Medline].

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Further Reading

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Keywords

anomalous left coronary artery from the pulmonary artery, ALCAPA, Bland-White-Garland syndrome, abnormal septation of the conotruncus into the aorta and pulmonary artery, persistence of the pulmonary buds together with involution of the aortic buds that form the coronary arteries, cardiac anomaly, myocardial ischemia, infarction of the anterolateral left ventricular free wall, congestive heart failure symptoms, CHF symptoms, heart defect, colic-like symptoms, coliclike symptoms, patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, coarctation of the aorta

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Contributor Information and Disclosures

Author

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Howard S Weber, MD, FAAP, FACC, FSCAI, Professor, Assistant Chief, Section of Pediatric Cardiology, Penn State University School of Medicine; Director, Pediatric Catheterization Laboratory, Milton S Hershey Medical Center
Howard S Weber, MD, FAAP, FACC, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Medical Editor

Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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