Familial Anomalous Origin of the Left Pulmonary Artery

Overview

Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) is a rare but serious congenital anomaly.

Causes

• Inheritance is not a factor. For example, if 2 family members are affected, the fact that they are within the same family did not have a role in their development of the condition.
• In utero exposure to teratogens, chromosomal abnormalities, or other risk factors are unrelated to ALCAPA.
• An isolated congenital cardiac defect, including patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, or coarctation of the aorta, rarely may be associated with ALCAPA. No specific association with any noncardiac anomalies is noted.

Diagnosis

ALCAPA is usually an isolated cardiac anomaly but, in rare incidences, has been described with patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, and coarctation of the aorta. Extremely rare variations of anomalous origin of the coronary arteries from the main pulmonary artery include the following:

• The left anterior descending or circumflex branches
• The right coronary, often discovered as an incidental finding on autopsy
• Both the right and left coronary arteries, a circumstance not compatible with survival