The single-stage extra-anatomic repair of an isolated interrupted aortic arch in an adult and a review of the literature

Abstract

Kesintiye uğramış arkus aort, çıkan ve inen aort arasında lümenin tamamen kaybı ve anatomik sürekliliğin olmaması ile karakterize, nadir ve ciddi bir doğuştan kalp defektidir. Bu malformasyonun prognozu, cerrahi olarak tedavi edilmez ise kötüdür. Bu yazıda tip A kesintiye uğramış arkus aortu olan ve cerrahi ile başarılı bir şekilde tedavi edilen 56 yaşında erkek hasta, konu ile ilgili literatür göz-den geçirilerek sunuldu.Anah tar söz cük ler: Erişkin; doğuştan; ekstra anatomik baypas; kesintiye uğramış arkus aort.Interrupted aortic arch is a rare and severe congenital heart defect characterized by a complete loss of luminal and anatomic continuity between the ascending and the descending aorta. This malformation has a poor prognosis without surgical treatment. In this article, we present a 56-year-old male patient with interrupted aortic arch type A who was treated successfully with surgery, and review the literature regarding this topic.Key words: Adult; congenital; extra-anatomic bypass; interrupted aortic arch.The interruption of the aortic arch (IAA) characterized by complete luminal and anatomic discontinuity between the ascending and descending aorta is an extremely rare entity in the adult population.[1] It is often diagnosed and repaired during the neonatal period. The prognosis for this anomaly depends on the associated congenital anomalies, but the outcome is usually very poor unless there is surgical treatment. We found 18 adult cases (18 years and older) of IAA which had been surgically treated by extra-anatomic bypass in the literature. Herein we describe a successful extra-anatomic repair in a patient who had an isolated type A aortic interruption. CASE REPORTA 56-year-old male patient arrived at the cardiology clinic of our hospital complaining of chest pain and shortness of breath. He also had a history of stroke due to an intracranial hemorrhage and hypertension, which had been treated with angiotensin-converting enzyme (ACE) inhibitors. On his physical examination, the peripheral pulses were normally palpable in the upper extremities, but they were reduced in the lower limbs. Cardiac auscultation revealed a rhythmic tachycardia with no additional heart sounds. Electrocardiography showed sinus tachycardia and signs of left ventricular hypertrophy and ST-T wave changes. Chest radiography showed an increased cardiothoracic ratio. Laboratory results were within normal ranges.An angiography was performed via the femoral approach for coronary artery imaging, but the catheter could not be inserted to the arcus aorta (Figure 1a). Interruption of the thoracic aorta was suspected. The angiography was then repeated via the brachial approach, and the interruption of the arcus aorta was diagnosed with normal coronary arteries (Figure 1b). An angiotomography of the aorta was then requested which disclosed an aortic occlusion 2 cm after the origin of the left subclavian artery (Figure 2a). This