Although echocardiography is the primary imaging technique for characterizing cardiac abnormalities, it is not the optimal method by which to evaluate the aortic arch and descending aorta. Interrupted aortic arch can be evaluated with use of echocardiography, CT, and magnetic resonance imaging (MRI). 6 In 1 study, overall survival at 16 years after study entry was found to be 59% in patients with interrupted aortic arch the survival rate increased to approximately 70% in patients who had undergone preoperative therapy and appropriate surgical techniques. 1 Type B interrupted aortic arch, major associated cardiac anomalies, low birth weight, and young age at presentation are the chief risk factors for death, and the associated cardiac anomalies are the main predictor of outcome. Although the mortality rate of patients with untreated interrupted aortic arch can exceed 90% from birth to age 1 year, the anomaly can infrequently present in adulthood if substantial collateral circulation develops. 2,6 Our asymptomatic patient had an isolated type A interruption. Genetic abnormalities, such as DiGeorge syndrome (chromosome 22q11.2 deletion syndrome), have been found in 50% to 80% of patients with interrupted aortic arch. In most patients, interrupted aortic arch is associated with additional cardiovascular anomalies, including ventricular septal defect, patent ductus arteriosus, bicuspid aortic valve, left ventricular outflow tract obstruction, aortopulmonary window, and truncus arteriosus. Type B interruption was most common (53%), followed by type A (43%) and type C (4%). In 1959, Celoria and Patton 5 classified interrupted aortic arch into 3 types, depending upon the site of discontinuity: distal to the left subclavian artery (type A), between the left common carotid and left subclavian arteries (type B), or between the brachiocephalic and left common carotid arteries (type C). The peak systolic pressure above the interrupted segment was 180 mmHg, and the pressure below it was 100 mmHg. Subsequently, aortography from the ascending aorta through the right brachial artery showed complete interruption of the aortic arch approximately distal to the origin of the left subclavian artery ( Fig. Because the patient's ascending aorta could not be reached through the right femoral artery, aortography was performed from the descending aorta ( Fig. Transthoracic echocardiography showed mild aortic insufficiency, aortic valve degeneration, and concentric left ventricular hypertrophy. A chest radiograph showed pectus excavatum. Results of electrocardiography were normal. A grade 2/6 midsystolic murmur was heard on the left side of the sternum at the 2nd intercostal space and the left scapular region in the back. The bilateral femoral and popliteal pulses were extremely weak, and the dorsalis pedis and anterior tibial pulses were impalpable. The pulses were equal over both upper extremities, and radial-femoral delay was noted. Upon his admission, the arterial blood pressure was 160/90 mmHg in both arms. In February 2010, a 19-year-old man was referred to our center for evaluation of hypertension and diminished lower-extremity pulses.

Interrupted aortic arch type