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Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA), Pediatric

Shellie M. Kendall, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

The anomalous coronary artery arises from the pulmonary artery rather than from its usual origin, the aorta. Most commonly, the anomalous left coronary artery arises from the pulmona...

DIAGNOSIS

HISTORY

  • Typically presents with paroxysms of poor feeding, pallor, cough, tachypnea, respiratory distress, and diaphoresis

  • Irritability, crying, appearance of being in pain (especially after m...

TREATMENT

GENERAL MEASURES

The first priority is to safely institute supportive care measures while expeditiously planning for surgical intervention. 

SURGERY/OTHER PROCEDURES

  • Direct reimplantation of th...

ONGOING CARE

PROGNOSIS

  • Untreated, 90% of those who present in infancy will die before the age of 1 year, usually at 1 to 2 months of age (when pulmonary vascular resistance falls).

  • Late results after su...

ADDITIONAL READING

  • Azakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricu...

CODES

ICD9

  • 746.85 Coronary artery anomaly

ICD10

  • Q24.5 Malformation of coronary vessels

SNOMED

  • 450301003 Anomalous origin of left coronary artery from pulmonary artery (disorder)

FAQ

  • Q: How do you differentiate crying from the symptoms of myocardial ischemia from crying from colic?

  • A: This is not easy, but clinical assessment should manifest the signs of CHF, shock, and low car...

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