J Extra Corpor Technol 2026, 58, 85--89 Ó The Author( s), published by EDP Sciences, 2026 https:// doi. org / 10.1051 / ject / 2025052
Available online at: ject. edpsciences. org
CASE REPORT
Perfusion strategy and surgical repair of Type A interrupted aortic arch and Type 1 aortopulmonary window: a case report
Paula Leigh( BA), Ed Harman( MS, CCP, FPP MBA), and S. Adil Husain( MD)
University of Utah Health, Primary Children’ s Hospital, Division of Cardiothoracic Surgery, Salt Lake City, UT, USA Received 14 April 2025, Accepted 2 September 2025
Abstract – Overview: Interrupted aortic arch in combination with an aortopulmonary window is an uncommon congenital cardiovascular defect. The anatomic separation between the aortic arch and descending aorta, combined with the communication between the ascending aorta and pulmonary trunk, leads us to a unique case where we can utilize the patient’ s anatomic defect towards an efficient perfusion strategy. Description: The patient was a 19-day-old neonate who was initially discharged home after birth with no suspicion of congenital heart disease. The patient was later diagnosed with a type A aortic interruption with a large type 1 aortopulmonary window. Due to the unique combination of defects, surgery was accomplished utilizing single instead of dual aortic cannulation, and the repair was accomplished using a double patch technique. Comment: An individualized perfusion strategy was employed by taking advantage of the defect.
Key words: Interrupted Aortic Arch, Aortopulmonary Window, Pediatric Perfusion, Congenital Heart Surgery, IAA, AP Window, IAA with AP Window.
Overview
Interrupted aortic arch with aortopulmonary window is an extremely rare congenital cardiovascular defect. An interrupted aortic arch( IAA) makes up only 1.5 % of all congenital cardiac patients [ 1 ]. This is an anatomical separation between the aortic arch and descending aorta and is classified secondary to the location of interruption. Distal perfusion is dependent on a patent ductus arteriosus, and thus patients receive a Prostaglandin E1 infusion following diagnosis [ 2 ]. The Celoria-Patton classification depicts the types of IAA based on the site of interruption. Type A interruption occurs just distal to the left subclavian artery, making up 30 % to 40 % of children with IAA [ 3 ]. Type B interruption occurs between the left carotid artery and the left subclavian( Figure 1)[ 4 ] and is the most common, making up approximately 53 % of IAA cases [ 3 ]. Type C interruption occurs between the innominate and left carotid arteries and is the rarest, occurring in only 4 % of IAA [ 3 ].
In conjunction with this condition, the prevalence of an aortopulmonary window( APW) makes for an exceedingly unusual combination. An APW is communication between the ascending aorta and the main pulmonary trunk. APW makes up less than 0.5 % of congenital cardiac defects [ 5 ] and is associated 50 % of the time with another defect [ 2 ]. The classification of APW has three categories based on the location of
* Corresponding author: ed. harman @ hsc. utah. edu the defect. Type 1 is most common, and describes the proximal defect located just above the sinus of Valsalva [ 6 ]. Type 2 describes the distal defect located in the most superior portion of the ascending aorta. Type 3 describes the total defect involving the majority of the ascending aorta( Figure 1)[ 4 ].
IAA is commonly associated with a ventricular septal defect( VSD), where blood shunts from the left to the right ventricle. IAA is also commonly associated with DiGeorge syndrome, or 22q11.2 deletion [ 1 ]. In its rare form, IAA with APW shunts oxygenated blood from the ascending aorta across the AP window through a patent ductus arteriosus to the lower body, using the defect as a conduit from the ascending to the descending aorta. In combination with an IAA with APW, an atrial septal defect( ASD) is often present in 65 % of cases [ 7 ]. With proper intervention, there is a very high chance of survival and a low chance of reoperation [ 7 ].
Description Patient history
The patient was a term infant female born at an outside hospital( OSH) via c-section for breech presentation. No anomalies were noted upon physical exam, and the patient was discharged home with the mother on day of life( DOL) 3 and described as an“ unremarkable normal newborn” in the hospital discharge summary. Patient presented to the emergency
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