Interrupted Aortic Arch

Jonas, Richard A.

doi:10.1002/9781118320754.ch15

Cited by 2 publications

(1 citation statement)

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“…Due to the frequent association of IAA with posterior malalignment ventricular septal defect (VSD) and subaortic stenosis, it was initially suggested that decreased flow in the ascending aorta during fetal life was the cause. 5 More recent evidence linking IAA with multiple genetic and neural crest cell anomalies has emerged. Chromosome 22q11 microdeletion, or DiGeorge syndrome, is present in up to 50% of patients with IAA and is the best known of these anomalies.…”

Section: Introductionmentioning

confidence: 99%

Perioperative and Anesthetic Considerations in Interrupted Aortic Arch

Burbano-Vera

Zaleski

Latham

et al. 2018

Semin Cardiothorac Vasc Anesth

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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.

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Section: Introductionmentioning

confidence: 99%