Perioperative and Anesthetic Considerations in Interrupted Aortic Arch

Abstract

Interrupted aortic arch (IAA) is defined as the loss of luminal continuity between the ascending and descending aorta and is classified based on the anatomic level of interruption. IAA is associated with a number of intracardiac anomalies with the most common being patent ductus arteriosus, ventricular septal defect, and left ventricular outflow obstruction. There is also a strong association between type B interruption and 22q11 deletion syndrome. The perioperative management of the neonate with IAA begins in the intensive care unit with optimization of end-organ perfusion and function. Survival depends on the prompt initiation of prostaglandin E1 in order to maintain ductal patency, careful management of the patient's ratio of pulmonary to systemic blood flow (Qp:Qs), and a thorough understanding of the physiologic implications of the surgical plan, type of interruption, and associated syndromes and anomalies. This review will focus on the anatomy, physiology, and perioperative anesthetic management considerations specific to the management of IAA.

Off-pump ascending aorta or aortic arch to descending aorta bypass with a pericardial roll for the treatment of critically ill infants with interrupted aortic arch

“…3,9 Bicuspid aortic valve and right subclavian artery abnormalities may also be seen in these patients. 1 Other associated rare cardiovascular anomalies include truncus arteriosus, singleventricle pathologies, atrioventricular canal defects and transposition of great arteries. 10 Various single-ventricle pathologies may be seen in 11% of patient with interrupted aortic arch.…”

“…8 In the neonatal period, the pathology is associated with severe congestive heart failure or lower body hypoperfusion, severe metabolic acidosis and multi-organ failure with the ductal closure. 1,3 The mortality in the first days of life is generally due to the closure of the patent ductus arteriosus; and without treatment, 90% of the patients die at a median age of 4 days. 3,5 The physical examination may reveal differential cyanosis and the gradient between lower and upper peripheral pulses in neonates 3,4 which are clues for either aortic interruption or coarctation which may be confirmed with transthoracic echocardiography, initially.…”

“…Care of the critically ill patients during or after cardiopulmonary bypass may be highly complicated due to the side effects of the extracorporeal circulation. 1 Additionally, the extensive mobilisation of the aortic arch may cause recurrent laryngeal and phrenic nerve injuries. 1 The residual aortic arch obstruction is not an uncommon pathology and ends up with requirement of the re-intervention in approximately 20% to 40% of the patients who receive aortic interruption repair.…”

“…Interrupted aortic arch is a rare congenital cardiac lession. 1 The incidence of the pathology is 3 per million live births and accounts for 1-1, 5% of all CHDs. 2 The patients with interrupted aortic arch present with poor perfusion in the distal part of the aorta and pulmonary over circulation in the early neonatal period.…”

“…2 The patients with interrupted aortic arch present with poor perfusion in the distal part of the aorta and pulmonary over circulation in the early neonatal period. 1 The pathology may be divided into three subtypes based on the location of the interruption such as Types A, B or C as the interruption distal to the left subclavian artery, the interruption distal to the origin of the left carotid artery and proximal to the origin of the left carotid artery, respectively. 3 The most common type is Type B with an incidence of 53% among cases followed by Type A (43%) and Type C (4%).…”