Which of the following anomalies is not associated with tetralogy of Fallot?

E. Primary pulmonary hypertension.

DISCUSSION: The first four defects listed occasionally are associated with tetralogy of Fallot. A right aortic arch is seen in 25% of patients with that lesion. Anomalous coronary arteries or a retroesophageal subclavian artery are found in as many as 5% to 10% of patients. Absence of a pulmonary artery is unusual but can present in as many as 3% of patients. Pulmonary hypertension is distinctly unusual with tetralogy of Fallot unless the patient has had excessive pulmonary blood flow from collaterals or systemic-to-pulmonary artery shunts for a long time. It is because these patients usually do not have pulmonary hypertension that infant correction with transannular patches can be performed with such great success.