Case Studies: Two Coronary Anomalies, Two Different Treatment Decisions

Case 1

A 13-year-old male was referred to the cardiology clinic for complaints of chest pain with exercise. His past medical history was significant for asthma and the chest pain had been attributed to the asthma. However, despite adequate asthma therapy, the chest pain continued to occur, prompting the referral. In clinic, the patient had a normal physical examination and resting ECG.

Because of the exercise-related symptoms, a transthoracic echocardiogram was performed. The echo demonstrated normal intracardiac anatomy and function but was suspicious for an anomalous aortic origin of the left main coronary artery from the right sinus of Valsalva (AAOLCA) with an interarterial course.

Cardiac MRI was performed and demonstrated AAOLCA with the left main coronary artery arising tangentially from the aorta, coursing between the aorta and pulmonary artery. There was no evidence of myocardial fibrosis or scar. The patient was then referred for an exercise stress test with nuclear perfusion. During the test, 3–4 millimeter ST segment depression in the infero-lateral leads was noted, suggestive of myocardial ischemia. The perfusion scan was also suspicious for exercise-induced ischemia. He did not develop symptoms during the test. Because of his anatomy and evidence concerning for myocardial ischemia, he was referred for surgical repair.

The patient’s surgery consisted of a modified unroofing procedure, performed using a median sternotomy. The anomalous coronary orifice was noted to be slit-like and arose above the left-right coronary commissure. The patient had an unremarkable postoperative course and was discharged home four days after surgery.

Postoperative evaluation

Postoperative echocardiograms have shown normal left and right ventricular systolic function with no wall-motion abnormalities and no aortic insufficiency. Exercise testing with nuclear perfusion three months after surgery revealed no evidence of ischemia. Cardiac MRI with adenosine perfusion performed six months after surgery revealed the left main coronary artery arose from the left (correct) sinus of Valsalva and was round and widely patent. There was no evidence of myocardial perfusion defects or scar.

Case 2

A 10-year-old girl was referred to the cardiology clinic because a new heart murmur was recently auscultated by her primary physician. She was asymptomatic. Her evaluation consisted of a physical examination, which was normal except for a flow murmur; a resting ECG, which was normal; and an echocardiogram. The echocardiogram demonstrated normal intracardiac anatomy and biventricular function, but was suspicious for an anomalous aortic origin of the right coronary artery from the left sinus of Valsalva (AAORCA) with an interarterial course. The patient subsequently underwent a cardiac MRI that confirmed AAORCA with an interarterial course. There was no evidence of myocardial scar or fibrosis.

An exercise stress test with nuclear perfusion was normal without evidence of myocardial ischemia. Since her diagnosis, the patient has remained asymptomatic. She is restricted from competitive athletics but enjoys jogging and playing intramural tennis. She has been followed routinely with echocardiograms and periodic exercise stress tests, all of which have demonstrated no evidence of myocardial ischemia. She has not been referred for surgical repair.

Significance

These case studies highlight that no two patients with a coronary anomaly are alike. The treatment and management depend on the coronary anatomy as well as symptoms and/or evidence of myocardial ischemia. In the above scenarios, our first patient had an interarterial AAOLCA, which is associated with a much higher risk of sudden cardiac death than AAORCA. The first patient also had evidence of myocardial ischemia on provocative testing, which is an indication for surgery. The second patient has remained asymptomatic without evidence of myocardial ischemia on testing and has continued to be managed nonsurgically.