Isolated Coronary Artery Anomalies Treatment & Management

Updated: Dec 18, 2014
  • Author: Jamshid Shirani, MD; Chief Editor: Eric H Yang, MD  more...
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Medical Care

The goal of medical therapy is to improve and preserve the hemodynamic status through acting on myocardial contractility, reducing congestion, and decreasing myocardial energy expenditure. Note the following:

  • Cardiac glycosides are a mainstay of inotropic therapy in those patients with congestive heart failure. Digoxin improves cardiac function because of its positive inotropic effect and negative chronotropic effect, but it must be used with caution in patients with myocardial ischemia.
  • Dobutamine (primarily a beta1-adrenergic agonist) is particularly useful for treatment of congestive heart failure in children because of its limited effect on heart rate and peripheral vasculature.
  • Phosphodiesterase inhibitors (ie, inamrinone, milrinone) can be used as alternatives to dobutamine because of their inotropic effect on the heart and the peripheral vasodilation that reduces the afterload. However, they should be avoided in patients with anomalous origin of a coronary artery from the pulmonary trunk because of the unpredictable vasodilatory effect of these agents on the pulmonary arterial system.
  • Loop diuretics (ie, furosemide) may be used in the presence of congestive heart failure.
  • Antibiotic prophylaxis for endocarditis is recommended for coronary artery fistulas.
  • Beta-adrenergic blocking agents have been used in isolated cases of symptomatic coronary artery anomaly to reduce myocardial oxygen demand and, thus, prevent ischemia.

The American Heart Association and the American College of Cardiology have published guidelines on the management of adult patients with congenital heart disease. [40]


Obtain consultations with a pediatric or adult cardiologist and a cardiothoracic surgeon.

Activity limitations

Discourage heavy exercise and competitive sports in patients with significant coronary artery anomalies at least until surgical correction is performed.


Surgical Care

Surgery is the only definitive treatment for coronary artery anomalies.

Coronary arteries originating from the pulmonary trunk are resected optimally from the pulmonary trunk and reimplanted into the ascending aorta. Alternative methods of revascularization include bypass grafts using the internal mammary artery or saphenous veins. In some patients with the origin of the coronary artery from the pulmonary trunk, an intrapulmonary tunnel may be produced to connect the ostium of the anomalous artery to the aorta.

Coronary artery fistulas can be treated with percutaneous transcatheter occlusion using a detachable balloon, detachable coils, double-umbrella devices, and microparticles of polyvinyl alcohol foam, or they can be treated surgically with a simple ligation. When possible, ligation is performed preferably at the point of entry of the coronary artery to the cardiac chamber. When this is not possible, ligation is performed internally. In patients with multiple lateral communications between the coronary artery and the cardiac chambers, a tangential arteriorrhaphy can be performed. The great risk in coronary ligation is postsurgical myocardial ischemia or infarction.

Coronary angioplasty with placement of stent is the treatment of choice for myocardial bridges if convincing evidence of myocardial ischemia exists. However, the vast majority of myocardial bridges do not appear to cause myocardial ischemia.

Coronary artery bypass grafting, preferably using the internal mammary artery, is the surgical treatment of choice for coronary artery atresia.