History

Most patients with coronary artery anomaly remain asymptomatic either because the anomaly does not produce any symptoms during life or because the first manifestation is sudden death. Anomalous coronary artery is recognized as the second most common cause of athletic field death among young competitive athletes.^[24]

In infants, myocardial ischemia may manifest as episodic crying, tachypnea, or wheezing. The infant may refuse to eat, presumably in order to avoid anginal pain.

In older individuals, symptoms are reported in less than 30% of patients before a diagnosis of coronary anomaly is made. These generally include palpitation, exertional dyspnea, angina or syncope, fatigue, or fever. These symptoms rarely raise clinical suspicion for diagnosis of coronary artery anomalies.

Physical Examination

Most coronary artery anomalies are discovered incidentally during noninvasive imaging, coronary angiography, or at autopsy and cause no clinical symptoms. However, particular subsets of these anomalies have been associated with sudden death, myocardial ischemia, congestive heart failure, or bacterial endocarditis.

Sudden death

This presentation has been observed in association with the origin of the left main or right coronary arteries from the opposite sinus of Valsalva and the type B (ie, between the aorta and pulmonary trunk) course of the anomalous vessel. This particular anomaly often is associated with a slitlike ostium and an obtuse takeoff of the proximal portion of the aberrant coronary artery. This combination may result in ischemia during exertion due to the stretching of the affected vessel that compromises blood flow at the ostium of the vessel. Increased cardiac output during exercise may also distend the ascending aorta and the pulmonary trunk and contribute to decreased blood flow through the anomalous coronary artery.

Sudden death also has been reported with congenital coronary artery structural abnormalities such as stenosis, hypoplasia, or atresia. Such structural abnormalities of the coronary arteries interfere with normal myocardial perfusion. Sudden death also has been reported in association with high takeoff of coronary arteries. The latter may lead to impairment of diastolic coronary artery flow. Ventricular fibrillation has been identified as the terminal event in some patients with coronary artery anomaly who have died suddenly during ambulatory electrocardiographic monitoring.

Myocardial ischemia

In addition to abnormalities mentioned under sudden death, myocardial ischemia also may occur in patients with anomalous origin of the left and, occasionally, right coronary artery from the pulmonary artery or right ventricle. In this type of anomaly, myocardial ischemia primarily occurs because of low coronary perfusion pressure secondary to the relatively low pulmonary diastolic pressure.

Myocardial ischemia also may occur in the setting of a single coronary artery when the aberrantly coursing vessel terminates prematurely and the myocardium distal to the vessel is inadequately perfused.

Intramyocardial course of coronary arteries (ie, myocardial bridge) occasionally has been associated with myocardial ischemia. The mechanism of myocardial ischemia in this condition is not fully elucidated.

Large coronary artery fistulas also may reduce myocardial perfusion and, thus, cause ischemia.

Congestive heart failure

Large coronary artery fistulas may result in right- or left-sided cardiac volume overload with or without symptoms of congestive heart failure. The hemodynamic effects of coronary artery fistulas depend on their site of drainage, diameter, and length. Drainage into the right heart produces left-to-right shunt with dilation of the right heart chambers and increase in pulmonary resistance. Eisenmenger syndrome has not been reported in association with such shunts. Drainage into the left heart produces left ventricular volume overload that may mimic aortic insufficiency clinically.

Heart failure also may be the predominant presentation in infants with the origin of the left main coronary artery from the pulmonary trunk. In the latter condition, the left ventricle appears dilated and globally hypokinetic on transthoracic echocardiography.

Bacterial endocarditis

Coronary artery fistulas may result in an increased risk of infective endocarditis or endarteritis depending on the location of the fistula. The infection commonly involves the receiving chamber of the heart at the entrance site of the anomalous coronary artery.

Signs

Physical findings generally are absent in most congenital coronary artery anomalies. The following signs may be present in patients with either anomalous origin of the left coronary artery from the pulmonary artery or a large coronary artery fistula:

Tachypnea and respiratory distress
Continuous precordial murmur
Systolic murmur of mitral regurgitation
S₃ or S₄ gallop rhythms
Cardiomegaly
Hepatomegaly
Edema
Peripheral cyanosis
Failure to thrive (infants)

Differential Diagnoses

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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).