How important are congenital anomalies of the coronary arteries?

Congenital coronary artery anomalies have been described with increased frequency due to the increased utilization of coronary angiography, but most are asymptomatic. Myocardial bridging due to an intramuscular course of a portion of the coronary artery (usually the left anterior descending) is the most common anomaly. In some patients, sufficient systolic compression of

the artery occurs to produce symptoms of ischemic heart disease (angina, myocardial infarction, sudden death). Ectopic aortic origin of either the right or left coronary artery is asymptomatic unless its course is altered to pass between the aorta and right ventricular outflow tract, which causes proximal obstruction of the artery and resultant ischemic symptoms. Anomalous origins from the pulmonary artery and congenital coronary arteriovenous fistulae usually become symptomatic during infancy and childhood.

1Are arrhythmias a problem in congenital heart disease?

Approximately one-quarter of patients with congenital heart disease have arrhythmias, especially patients who have undergone corrective intracardiac surgery. More than 50% of patients with corrected transposition, 30% with tetralogy of Fallot, 25% with Fontan repair, and 10% with ventricular or atrial septal defect have postoperative arrhythmias. Supraventricular arrhythmias are most common, but sudden death due to ventricular arrhythmias is believed to occur in 5-10% of these patients.

1Ventricular arrhythmias are most common after repair of tetralogy of Fallot. What are the risk factors for sudden death in these patients?

Older age at the time of repair

Prolonged postoperative recovery

Right ventricular end-systolic pressure 60 mmHg

Right ventricular end-diastolic pressure 10 mmHg

Depressed right ventricular systolic function

Significant tricuspid or pulmonic regurgitation

Fifteen percent of postoperative patients have inducible ventricular tachycardia during electrophysiologic testing.

Describe the features of Eisenmenger’s syndrome. What is Eisenmenger’s complex? Eisenmenger’s syndrome is a broad term applied to any anomaly in which the pathophysiologic process (e.g., increased pulmonary blood flow) leads to obliterative pulmonary vascular disease with resultant pulmonary hypertension whether or not there is an associated right-to-left shunt and cyanosis. Eisenmenger’s complex refers to patients with congenital heart disease involving shunts with pulmonary hypertension severe enough to cause reversal of a left-to-right shunt with resultant right-to-left shunting through the defect. Eisenmenger originally described patients with ventricular septal defects. Pulmonary hypertension due to Eisenmenger-type pathophysiology is usually irreversible but may improve after corrective surgery if the associated pulmonary vascular anatomic changes are not too severe.

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