Anomalous Left Coronary Artery From the Pulmonary Artery Treatment & Management

  • Author: Mary C Mancini, MD, PhD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Dec 1, 2011
 

Medical Care

  • Initial management of anomalous left coronary artery from the pulmonary artery (ALCAPA) is both supportive and temporary. Treatment of congestive heart failure (CHF) includes carefully using diuretics, afterload reduction medications, and inotropic drugs.
  • Although systemic oxygen transport may be reduced in the presence of low systemic blood flow, using 100% oxygen may be deleterious. Oxygen may further reduce pulmonary vascular resistance and magnify coronary steal from the right coronary artery into the pulmonary arteries.
  • A similar phenomenon occurs with aggressive afterload reduction, during which right coronary artery perfusion may be reduced, leading to decreased left coronary blood flow.
  • Inotropic support, on the other hand, may significantly increase myocardial oxygen consumption, which, in the presence of reduced myocardial blood flow, may result in worsening ischemia.
  • Increasing reports of catheter intervention for this lesion are emerging. The results in these instances remain conflicting. Surgical intervention remains the procedure of choice.
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Surgical Care

  • Spontaneous resolution of CHF symptoms is rare. Surgical revascularization of the left coronary artery system is usually necessary.
  • Once the patient is stabilized, perform surgical revascularization to create a 2 coronary artery system. Over the years, the following techniques have been advocated:
    • Ligation of the left coronary artery at its origin from the main pulmonary artery is an original technique, performed without the use of cardiopulmonary bypass. The long-term results were not optimal since myocardial perfusion remained solely dependent on extensive collateralization from the right coronary artery, and the patient remained at risk for ischemic episodes and sudden death.
    • Current surgical procedures are directed at establishing revascularization by creating a 2 coronary artery system via either (1) a left subclavian artery-coronary artery anastomosis, (2) a saphenous vein bypass graft, (3) Takeuchi procedure (creation of an aortopulmonary window and an intrapulmonary tunnel extending from the anomalous ostium to the window), or (4) direct reimplantation.[9] By establishing a patent 2 coronary artery system, most patients experience normalization of left ventricular systolic function, thereby improving long-term survival.
    • The need for simultaneous mitral valve reconstruction, in the presence of severe insufficiency, is controversial because spontaneous improvement of mitral valve function often occurs following surgical revascularization.
    • Once revascularization to a 2 coronary artery system is accomplished, most patients demonstrate improved left ventricular systolic function, decreased mitral valve insufficiency, and resolution of CHF symptoms. In many cases, the classic infarct pattern on electrocardiography eventually disappears following normalization of left coronary blood flow (see the image below). Occasionally, persistent refractory mitral regurgitation will necessitate delayed mitral valve repair or replacement. Electrocardiogram in 2-month-old infant with anomaElectrocardiogram in 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery 17 months following successful surgical revascularization, demonstrating complete resolution of the anterolateral infarction pattern and ST-T wave changes.
  • Postoperative care, precautions, and complications
    • Diuretics, and afterload reduction may be necessary until there is significant improvement in left ventricular systolic and diastolic function with resolution of mitral valve insufficiency. These medications improve cardiac output and eliminate the preoperative symptoms of congestive heart failure.
    • Although unusual, there remains a risk of cardiac dysrhythmia secondary to preoperative myocardial ischemia or infarction. Monitor continuously in the immediate postoperative period.[4, 10, 11, 12]
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Consultations

Pediatric cardiologist; Pediatric cardiothoracic surgeon

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Diet

No specific postoperative restrictions are usually necessary.

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Activity

  • Restrictions are directly related to the severity of left ventricular dysfunction and postoperative mitral valve insufficiency.
  • No specific recommendations are necessary since the majority of patients are infants. For patients who are able to participate in exercise or competitive sports or those with residual postoperative hemodynamic problems, consider recommending avoidance of significant isometric activities.
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Contributor Information and Disclosures
Author

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

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

Specialty Editor Board

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.

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.

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z 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.

References

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Preoperative electrocardiogram in a 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery demonstrating pathologic Q waves in leads I and aVL and diffuse ST-T wave changes consistent with an anterolateral infarction.
Electrocardiogram in 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery 17 months following successful surgical revascularization, demonstrating complete resolution of the anterolateral infarction pattern and ST-T wave changes.
Two-dimensional echocardiographic image (parasternal short axis view) in a patient with anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). The left coronary artery (white arrow) appears to course towards the main pulmonary artery (MPA) just above the pulmonary valve and not to the aortic root (Ao). RV = Right ventricle.
Two-dimensional echocardiographic image with color flow mapping (parasternal short axis view) in the same patient with anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). The addition of color flow mapping to the 2-dimensional image demonstrates abnormal flow reversal within the left coronary artery (white arrows) towards the main pulmonary artery (MPA) just above the pulmonary valve. RV = Right ventricle. Ao = Aortic root.
Doppler interrogation of the abnormal color flow jet is depicted, demonstrating abnormal flow within the main pulmonary artery towards the transducer in diastole, which represents runoff from the anomalous left coronary artery (large white arrowhead). Small white arrow: Normal antegrade main pulmonary artery flow in systole. MPA = Main pulmonary artery.
Aortogram in a patient with suspected anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Frontal (left panel) and lateral (right panel) images demonstrating an enlarged right coronary artery (small white arrow), which fills a small left coronary system (solid arrow head) via collaterals with eventual faint opacification of the main pulmonary artery (not demonstrated in this frame).
Main pulmonary artery angiogram demonstrating the technique of stop flow angiography. There is retrograde opacification of the entire left coronary artery system, which originates from the distal main pulmonary artery (MPA), including the anterior descending (solid white arrowhead) and circumflex (small white arrow) branches. Left panel: Frontal image. Right panel: Lateral image.
 
 
 
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