It is Not a Broken Heart; It Just Looks Different!

Author: Keith Bullinger, MD, Additional Authors^**

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Introduction: Anomalous aortic origin of a coronary artery is an uncommon condition associated with acute coronary syndrome and sudden cardiac death in healthy individuals, but the variable anomalous courses of the arteries are frequently benign. This case raises awareness of this rare finding and offers a reminder that anatomic anomalies do not always require intervention unto themselves.

Case Presentation: A 64-year-old female presented to the emergency department for one hour of severe, progressively worsening exertional angina radiating to her left arm and back, with associated diaphoresis, dizziness, and nausea. Medical history included obesity, Roux-en-Y gastric bypass, deep vein thrombosis, hypertension, hyperlipidemia, and total thyroidectomy for papillary thyroid carcinoma. Medications were aspirin, simvastatin, levothyroxine, and losartan. Family history was significant for death secondary to myocardial infarction in both parents.

On presentation, blood pressure was 169/103 mmHg and pulse was 50 beats/minute. Physical examination was unremarkable. Initial troponin I was 0.017 ng/mL (normal < 0.120 ng/mL) and peaked to 8.651 ng/mL nine hours later. A complete laboratory work-up revealed no other significant abnormalities. Serial electrocardiograms indicated dynamic T-wave changes in leads III and aVF.

Initial treatment included aspirin, clopidogrel, heparin, and metoprolol. An echocardiogram showed normal ejection fraction with moderate hypokinesis of the mid inferolateral, basal inferior, and mid inferior walls. Coronary angiography demonstrated an anomalous left coronary artery (LCA) arising from the right aorta and appearing to run anterior to the pulmonary artery. The right coronary artery (RCA) was large with two posterior descending arteries and severe diffuse distal disease in two dominant posterolateral small branches. CT angiography of the coronary arteries confirmed that the LCA was split from the RCA, originating independently over the right coronary cusp, and following a prepulmonic course. The aortic valve was normal trileaflet and the aorta was non-aneurysmal. She received no intervention and medications were optimized prior to discharge. She remained well on follow-up.

Discussion: Anomalous coronary artery is second only to hypertrophic myopathy as cause of non-traumatic sudden cardiac death. Numerous anomalies of the coronary arteries have been described, with RCA anomalies being 10-times more common than LCA anomalies. Intramural aortic course or a course between the aorta and pulmonary artery portend a worse prognosis. Hence, confirmation of course is crucial and non-invasive testing with CT angiography of the coronary arteries may be superior to invasive angiography in delineating the course and identifying high risk abnormalities that might benefit from surgical intervention. Patients with such anomalies may present at young ages with unstable angina, shortness of breath, syncope, arrhythmias, or sudden death. Recognition and characterization of these anomalies remains important to identify those at greater risk for sudden cardiac death to optimize medications and lifestyle modifications and institute surgical referral if necessary.

Conclusion: This patient’s prepulmonic course of the anomalous LCA is likely benign and unrelated to her myocardial infarction. Her case reassures that anomalous coronary arteries may remain undetected throughout a patient’s lifetime, do not necessarily require invasive intervention, and may be spared of atherosclerosis when non-anomalous coronaries are diseased.

**Additional Authors:

Keith Bullinger, MD, CWRU – MetroHealth, Cleveland, Ohio

Srinivasa Potla, MD, CWRU – MetroHealth, Cleveland, Ohio

Akram Alkrekshi, MD, CWRU – MetroHealth, Cleveland, Ohio

Aisha Siraj, MD, CWRU – MetroHealth, Cleveland, Ohio