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Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery Workup

  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Jonah Odim, MD, PhD, MBA  more...
 
Updated: Jan 30, 2015
 

Laboratory Studies

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  • Perform arterial blood gas measurements, including an assessment for acidosis and carbon dioxide retention, in the setting of respiratory distress.
  • Cardiac enzymes (eg, troponin I, creatine kinase–MB fraction) may be elevated in patients with myocardial ischemia, but results are not specific for anomalous left coronary artery from the pulmonary artery (ALCAPA).
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Imaging Studies

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  • Chest radiography: Chest radiography reveals cardiomegaly, left atrial and left ventricular enlargement, and pulmonary venous congestion.
  • Echocardiography
    • Currently, most cases of anomalous left coronary artery from the pulmonary artery can be diagnosed by echocardiography. In infants presenting with left ventricular dilation and dysfunction, special attention should be directed to the coronary artery anatomy during echocardiographic evaluation.
    • Two-dimensional (2D) imaging alone is usually inadequate to thoroughly evaluate for anomalous left coronary artery from the pulmonary artery. The anomalous coronary may course very close to the aortic sinus and create the false impression of a normal anatomic origin of the left coronary artery. Usually, 2D imaging identifies an enlarged right coronary artery at its origin and proximal course. Coupled with color-flow Doppler imaging, 2D imaging greatly increases the diagnostic findings of echocardiography.
    • Color-flow Doppler imaging demonstrates abnormal retrograde flow in the anomalous left coronary artery and into the main pulmonary artery segment. The color flow into the pulmonary artery should not be confused with a shunt from a ductus arteriosus or a coronary-cameral fistula.
    • Transesophageal echocardiography may be useful in the rare adult patient in whom anomalous left coronary artery from the pulmonary artery is suspected, but this examination is usually unnecessary in infants.
    • Coronary CT angiography can reveal anomalous left coronary artery from the pulmonary artery in adult patients; however, it does not eliminate the need for cardiac catheterization.
    • Examples of echocardiography findings are shown in the images below.
      Parasternal long-axis 2-dimensional echocardiogramParasternal long-axis 2-dimensional echocardiogram view of the pulmonary artery. The anomalous left coronary artery and first order branches of the anomalous left coronary artery (LCA) are identified.
      Parasternal long-axis 2-dimensional echocardiogramParasternal long-axis 2-dimensional echocardiogram. Very dilated left ventricle with mitral regurgitation.
      Parasternal long-axis 2-dimensional, color-flow DoParasternal long-axis 2-dimensional, color-flow Doppler echocardiogram. Normal flow in the pulmonary artery. Abnormal retrograde flow (*) in the anomalous left coronary artery from the pulmonary artery (ALCAPA).
      Parasternal short-axis 2-dimensional, color-flow DParasternal short-axis 2-dimensional, color-flow Doppler echocardiogram. Normal antegrade flow in the proximal right coronary artery.
      Modified parasternal long-axis echocardiogram withModified parasternal long-axis echocardiogram with color-flow Doppler. Abnormal retrograde flow in the left anterior descending (LAD) coronary artery.
      Apical 4-chamber 2-dimensional echocardiogram. NotApical 4-chamber 2-dimensional echocardiogram. Note the very dilated left atrium and left ventricle.
  • Ultrasonography: Examples of ultrasonography in anomalous left coronary artery from the pulmonary artery are shown in the images below.
    Intraoperative transesophageal, transverse plane, Intraoperative transesophageal, transverse plane, 4-chamber view, 2-dimensional, color-flow Doppler ultrasound image. Note the dilated left atrium, dilated left ventricle, and mitral regurgitation. LV=left ventricle; RV=right ventricle.
    Intraoperative transesophageal, transverse plane, Intraoperative transesophageal, transverse plane, 2-dimensional ultrasound image. Main pulmonary artery with origin of the anomalous left coronary artery. Note the first-order branching into the left anterior descending and circumflex coronary arteries.
    Intraoperative transesophageal, transverse plane, Intraoperative transesophageal, transverse plane, 2-dimensional, color-flow Doppler ultrasound image. Main pulmonary artery with origin of the anomalous left coronary artery. Abnormal retrograde flow is noted in the left anterior descending (LAD) coronary artery.
    Intraoperative transesophageal, transverse plane, Intraoperative transesophageal, transverse plane, 2-dimensional ultrasound image. Completed repair of the left main coronary artery (LMCA) anastomosed to the aorta. LAD=left anterior descending coronary artery.
    Intraoperative transesophageal, transverse plane, Intraoperative transesophageal, transverse plane, 2-dimensional, color-flow Doppler ultrasound image. Completed repair with normal antegrade flow in the circumflex and left anterior descending (LAD) coronary arteries. LMCA=left main coronary artery.
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Other Tests

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  • Electrocardiograms can reveal an infarct pattern, typically in an anteroseptal distribution. Wide and/or deep Q waves are typically present in leads I and aVL. Loss of normal R-wave progression in the precordial leads and T-wave inversion in leads I, aVL, and the left precordial leads may be observed.
  • The electrocardiogram changes noted above are nonspecific for anomalous left coronary artery from the pulmonary artery and may be encountered in other forms of cardiomyopathy.
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Diagnostic Procedures

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  • If the diagnosis is unclear, cardiac catheterization and angiography may be indicated to definitively evaluate the coronary arteries.
  • Typically, right ventricular, pulmonary artery and left ventricular end-diastolic, and pulmonary artery wedge pressures are increased. A small shunt (Qp/Qs of approximately 1-1.5) may be present.
  • Angiography images delineate the anomalous left coronary artery from the pulmonary artery. Aortic root, left ventricular, and balloon occlusion angiography of the pulmonary artery can be used to delineate the anatomy in patients with anomalous left coronary artery from the pulmonary artery.
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Contributor Information and Disclosures
Author

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher Mart, MD Associate Professor, Pediatric Echocardiography, Department of Pediatrics, Division of Pediatric Cardiology, University of Utah, Primary Children's Medical Center

Christopher Mart, MD is a member of the following medical societies: American College of Cardiology, American Society of Echocardiography, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

John Myers, MD Director, Pediatric and Congenital Cardiovascular Surgery, Departments of Surgery and Pediatrics, Professor, Penn State Children's Hospital, Milton S Hershey Medical Center

John Myers, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, American Medical Association, Congenital Heart Surgeons Society, Pennsylvania Medical Society, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Additional Contributors

Daniel S Schwartz, MD, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, FACS is a member of the following medical societies: Society of Thoracic Surgeons, Western Thoracic Surgical Association, American College of Chest Physicians, American College of Surgeons

Disclosure: Nothing to disclose.

References
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A 3-month-old child presenting with anomalous left coronary artery from the pulmonary artery (ALCAPA). Note the large prominent Q waves in leads I, aVL, and V4-V6.
Parasternal long-axis 2-dimensional echocardiogram view of the pulmonary artery. The anomalous left coronary artery and first order branches of the anomalous left coronary artery (LCA) are identified.
Parasternal long-axis 2-dimensional echocardiogram. Very dilated left ventricle with mitral regurgitation.
Parasternal long-axis 2-dimensional, color-flow Doppler echocardiogram. Normal flow in the pulmonary artery. Abnormal retrograde flow (*) in the anomalous left coronary artery from the pulmonary artery (ALCAPA).
Parasternal short-axis 2-dimensional, color-flow Doppler echocardiogram. Normal antegrade flow in the proximal right coronary artery.
Modified parasternal long-axis echocardiogram with color-flow Doppler. Abnormal retrograde flow in the left anterior descending (LAD) coronary artery.
Apical 4-chamber 2-dimensional echocardiogram. Note the very dilated left atrium and left ventricle.
Intraoperative transesophageal, transverse plane, 4-chamber view, 2-dimensional, color-flow Doppler ultrasound image. Note the dilated left atrium, dilated left ventricle, and mitral regurgitation. LV=left ventricle; RV=right ventricle.
Intraoperative transesophageal, transverse plane, 2-dimensional ultrasound image. Main pulmonary artery with origin of the anomalous left coronary artery. Note the first-order branching into the left anterior descending and circumflex coronary arteries.
Intraoperative transesophageal, transverse plane, 2-dimensional, color-flow Doppler ultrasound image. Main pulmonary artery with origin of the anomalous left coronary artery. Abnormal retrograde flow is noted in the left anterior descending (LAD) coronary artery.
Intraoperative transesophageal, transverse plane, 2-dimensional ultrasound image. Completed repair of the left main coronary artery (LMCA) anastomosed to the aorta. LAD=left anterior descending coronary artery.
Intraoperative transesophageal, transverse plane, 2-dimensional, color-flow Doppler ultrasound image. Completed repair with normal antegrade flow in the circumflex and left anterior descending (LAD) coronary arteries. LMCA=left main coronary artery.
(1) Cardioplegia catheter in ascending aorta. (2) Cross-clamp on ascending aorta. (3) Cross-clamp on main pulmonary artery. (4) Arterial bypass cannula in the main pulmonary artery. (5) Cardioplegia catheter in the main pulmonary artery. (6) Dilated conal branch of the right coronary artery. (7) Venous bypass cannula in the right atrial appendage. (8) Left heart vent.
(1) Transverse anterior incision in the main pulmonary artery trunk. (2) Probe is in the orifice of the anomalous left coronary artery.
(1) Divided distal main pulmonary artery. (2) Left coronary artery button. (3) Divided proximal main pulmonary artery.
(1) Left coronary artery button. (2) Divided proximal main pulmonary artery. (3) Bypass sucker in transverse aortotomy (to visualize the aortic sinuses). (4) Incision in aortic sinus for site of aortocoronary anastomosis.
(1) Completing the anastomosis of the left coronary artery to the aortic sinus. (2) Divided proximal main pulmonary artery.
(1) Completed anastomosis of the left coronary artery to the aortic sinus. (2) Divided proximal main pulmonary artery. (3) Ascending aorta, transverse aortotomy.
(1) Suture closure of the aortotomy.
(1) Distal divided main pulmonary artery. (2) Beginning re-anastomosis (posterior wall) of the main pulmonary artery. (3) Proximal main pulmonary artery.
(1) Completed repair of the main pulmonary artery re-anastomosis.
 
 
 
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