Latent Congenital Defect: Interrupted Aortic Arch in an Adult—Case Report and Literature Review

Abstract

Interrupted aortic arch (IAA) is rare congenital cardiac defect defined as a complete loss of luminal and anatomical continuity between ascending and descending segments of the aorta. Usually it is detected in the perinatal period or during the first hours or days of infancy. If not treated surgically, it usually is lethal. Nevertheless, diagnosis can be made in adults but is a very rare entity. Extremely few cases in adults are reported in the pertinent medical literature. We present an asymptomatic 62-year-old patient who was found to have IAA after examination for hypertension. The patient underwent a successful anatomical repair, with an uneventful postoperative course, and follow-up examinations reveal regression of hypertension and excellent health condition.

Aortic Interruption Presenting with Recurrent Ischemic Strokes in an Adult

“…For surgical repair of IAA type A, interposition of a graft between left subclavian artery and descending thoracic aorta has become the standard approach since its first description in 1961. Our case demonstrates once again that this procedure can successfully be performed in adulthood, like prior adult cases in the literature 3,4 . Quick and excellent regression of collateral network following surgical correction, as demonstrated nicely in the presented case, is an early sign of therapeutic success.…”

“…Of note, patients with at least one of the accompanying cardiac defects like patent ductus arteriosus, truncus arteriosus, ventricular septal defect, bicuspid aortic valve, left ventricular outflow tract obstruction, aberrant innominate arteries, and aortopulmonary window, which are reported in up to 98% of IAA patients, become symptomatic earlier, whereas patients devoid of these abnormalities survive without a treatment for a longer period 10,11 . In adult cases, IAA usually presents with secondary hypertension or its complications 4,11 . Rarely, IAA can be detected as an incidental finding 12 .…”

“…Diagnosis of an IAA after adolescence is exceptional. In less than 50 adult cases with IAA reported in the literature, only seven were older than 50 years of age 3,4 . In these adult cases, clinical manifestations were usually related to hypertension, and included 3 patients with aneurysmal subarachnoid hemorrhage, 5–7 which is a rare but well‐recognized manifestation of aortic coarctation 8 .…”

An Extremely Unusual Case of Type A Interrupted Aortic Arch in a Young Male

“…6 Interruption is very rarely encountered in adult patients. To the best of our knowledge, there have been around 25 cases only reported in world literature 7,8 indicating the rarity of this anomaly. In the few reported adult cases, clinical symptoms vary from normal to weakness, hypertension, malaise, headache, differences in blood pressures in the limbs, swelling of limbs and ischaemic changes in the lower limbs.…”

Interrupted Aortic Arch in the Adult

“…Another factor, which further complicates the study of IAA in adults, is the type of treatment (medical versus surgical) used in these patients. For example, Sakellaridis et al, 5 limiting their literature search to the reports in which the patients underwent surgery, quoted a total of only 21 patients.…”