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

Anomalous Left Coronary Artery From the Pulmonary Artery: Follow-up

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

Follow-up

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.

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.

Inpatient & Outpatient Medications

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

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.

Prognosis

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

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.

Miscellaneous

Medicolegal Pitfalls

  • The most serious, yet avoidable, medicolegal complication is the mistaking of symptoms in the patient older than several months who presents with anomalous left coronary artery from the pulmonary artery (ALCAPA) as idiopathic dilated cardiomyopathy or end stage myocarditis. The initial presentation may be entirely similar but the treatments are dramatically different. The clinical and initial laboratory findings may be remarkably similar. If there is significant pulmonary artery hypertension, the diagnostic ECG findings may not be apparent.
  • A high index of suspicion is necessary to make the diagnosis of ALCAPA, since surgical treatment is extremely successful and results in an excellent long-term prognosis.

Special Concerns

  • With successful surgical revascularization and resolution of myocardial dysfunction in female patients, pregnancy and normal vaginal delivery are not contraindicated.
  • In patients with persistent myocardial dysfunction, mitral valve insufficiency, or coronary ischemia, the ability to carry a pregnancy to term varies. These patients should be considered high risk and require close observation throughout the pregnancy by both a cardiologist and a perinatologist because the increase in blood volume may be deleterious to the patient's hemodynamic status. A normal vaginal delivery may also be contraindicated in this situation.
 


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References
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References

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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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