Case Presentation:

We report here a case of a 33 year old African American female with a history of neurofibromatosis, who presented for an elective cardiac catheterization after having an abnormal stress test showing an apical perfusion defect. The left main coronary artery was not able to be cannulated on cardiac catheterization. The left anterior descending and circumflex arteries filled retrograde from a right coronary artery system. A cardiac CT revealed an anomalous left main coronary artery originating from the main pulmonary artery. It also revealed an incidental right upper lung lobe mass. Follow up chest CT revealed a large right para-spinal mass consistent with neurofibroma confirmed on a cardiac MRI. An ECHO revealed moderate LV dysfunction and mild mitral regurgitation. The patient responded well to medical therapy and was followed up at an adult congenital heart clinic. She will undergo elective surgery with either a coronary re-implantation or coronary artery bypass grafting with ligation.


Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital abnormality that affects 1 out of every 300,000 live births. It typically presents in infants as heart failure and mitral valve insufficiency. However, in some cases due to effective collateral circulation, survivors may be asymptomatic till early adulthood. Adults with ALCAPA present with severe dilated cardiomyopathy, malignant ventricular arrhythmias, sudden cardiac death or myocardial infarction.

Conclusions: This case demonstrates a rare congenital heart abnormality in a patient with neurofibromatosis. Multi-modality imaging, in particular, coronary angiography and cardiac CT played a crucial role in the diagnosis of this extremely rare congenital defect.