Anomalous Left Coronary Artery From the Pulmonary Artery Follow-up

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

Further Inpatient Care

  • The severity of symptoms in patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) at presentation determines whether the patient is admitted to an intensive care unit (ICU) for aggressive medical management of congestive heart failure (CHF) before surgical revascularization.
  • Initial postoperative management occurs in a pediatric ICU until the patient is extubated and no longer requires intravenous inotropic support or antiarrhythmics.
  • Following surgical revascularization, postoperative care includes the use of inotropes, diuretics, and afterload reduction medication to improve cardiac output and to eliminate the preoperative symptoms of CHF.
  • Monitor continuously during the immediate postoperative period because there is a risk, although unusual, of cardiac dysrhythmia secondary to preoperative myocardial ischemia or infarction.
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Further Outpatient Care

  • The clinical status of the patient, in relation to residual CHF symptoms, determines the frequency of postoperative outpatient follow-up visits.
  • Most patients do not require frequent cardiac evaluation following surgical revascularization once ventricular function and mitral valve insufficiency is dramatically improved.
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Inpatient & Outpatient Medications

  • The short-term use of oral digoxin, diuretics, and ACE inhibitors is common following surgical revascularization.
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Complications

  • Complications are rare. The need for future valve surgery depends on the occurrence of hemodynamic complications (eg, residual mitral valve insufficiency precipitated by permanent damage of the mitral valve architecture) following surgery.
  • Late complications related to coronary artery insufficiency are more likely to occur if revascularization was accomplished by any of the following:
    • Surgical ligation
    • Bypass grafts that may become occluded or stenotic
    • Intrapulmonary tunnel technique, which may cause supravalvar pulmonary stenosis or, less commonly, become obstructed at the surgically created aortopulmonary window
  • Inadequate growth of the coronary anastomosis is possible, although unlikely, if surgical reimplantation of the left coronary artery was performed. This occurrence is similar to the rare reports of late coronary artery problems following the arterial switch procedure for transposition of the great vessels that also requires direct coronary transfer and reimplantation.
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Prognosis

  • Early diagnosis using echocardiography with color flow mapping and improvements in surgical techniques (eg, myocardial preservation) dramatically improve prognosis.
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Patient Education

  • All patients should undergo formal exercise stress testing at an appropriate age as an aid in determining an appropriate exercise program.
  • Long-term physical restrictions, including restrictions of participation in competitive sports, are a direct function of whether myocardial ischemia is evident at rest or during exercise.
  • No dietary restrictions are necessary following successful surgical revascularization with subsequent clinical improvement.
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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.

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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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