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 in patients with 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.

A 2018 case report discussed a middle-aged patient with isolated single coronary artery with absent right coronary artery who presented with unstable angina and underwent successful conservative management.^[45] Aside from symptomatic complaints of pain, palpitations, and dizziness, the patient's vital signs, physical examinations, and transthoracic echocardiographic findings were unremarkable; electrocardiography revealed normal sinus rhythm with intermittent sinus bradycardia and nonspecific T-wave changes, and selective coronary angiography and aortography showed unusual cardiac features.^[45]

The American Heart Association and the American College of Cardiology have published guidelines on the management of adult and older adult patients with congenital heart disease.^{[46, 47]}

Consultations

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

Activity limitations

Discourage strenuous physical activity such as heavy exercise and competitive sports in patients with significant coronary artery anomalies and those with symptoms of myocardial ischemia 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. A 2018 case study described successful direct surgical implantation of the right coronary artery into the aorta in a 2-month-old infant with isolated anomalous origin of the right coronary artery from the main pulmonary artery, with good outcome at 7 months follow-up.^[19]

For anomalous coronary arteries that course in between the aortic root and the pulmonary trunk and have resulted in myocardial ischemia or sudden cardiac death, surgical intervention is recommended. In addition to relocation of the coronary ostium to the appropriate anatomic location, other surgical techniques have been employed. These alternative methods of revascularization have included unroofing and bypass grafts using the the internal mammary artery or saphenous veins. Current evidence indicates that unroofing may be a safe and effective surgical approach in such cases.^[48]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.

In a study of data from records of 18 patients who underwent transcatheter closure (TCC) approaches for coronary artery fistulas, investigators found that the choices of TCC technique and device selection varied and were mainly determined by the anatomic type of the fistula.^[49]

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.

In a case study of a 7-week-old neonate, investigators described left main coronary artery atresia (LMCAA) revascularization with a left internal mammary artery (LIMA) graft and mitral valve repair. This procedure had a successful outcome 1 year postoperatively.^[50]

Medication

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Media Gallery

Coronary angiography showing separate origin of the left anterior descending (LAD) and left circumflex (LCx) coronary arteries from the left coronary sinus of the aorta (absent left main).
Coronary angiography showing the anomalous origin of the left main (LM) coronary artery from proximal right coronary artery (RCA) with subsequent retroaortic (dorsal [type D]) course to the left side.
Selective left coronary artery angiogram demonstrating anomalous origin of obtuse marginal (OM) coronary artery from proximal left anterior descending (LAD) coronary artery. LM=left main, LCx=left circumflex.
Coronary angiography showing the anomalous origin of the right coronary artery (RCA) from the left anterior descending (LAD) coronary artery with subsequent anterior course (anterior [type A]) to the right atrioventricular groove.
Coronary angiography showing the origin of the right coronary artery (RCA) as the continuation of the left circumflex (LCx) coronary artery.
Coronary angiography showing the presence of a fistula originating from a diagonal (diag) branch of the left anterior descending coronary artery with anomalous communication with the pulmonary artery (PA).