88 P. Leigh et al.: J Extra Corpor Technol 2026, 58, 85--89
Figure 4. Flow pattern post cannulation through the defect.
switched to a low-flow nasal cannula. The patient was discharged home on post op day 11.
Comment
Figure 3. A. Echocardiogram identifying large APW. B. Echocardiogram illustrating flow through APW.
The MPA and RPA were reconstructed at the site of the APW using a large patch of pulmonary homograft material. With the left heart vented for deairing purposes, the patient was placed in the Trendelenburg position, and the cross clamp was removed. The heart regained normal sinus rhythm. After adequate rewarming, the patient was weaned from CPB and separated without issue. Modified ultrafiltration was performed, and protamine was subsequently delivered to achieve hemostasis.
Total CPB time was 156 min with an aortic cross-clamp time of 84 min. Upper extremity only flow time was 12 min with 44 min of low flow antegrade cerebral perfusion.
The patient returned to the Cardiac ICU with an open chest in stable condition. The patient’ s lactate returned to normal, and on post op day 2, she underwent delayed sternal closure with no complications. On post op day 3, she was extubated and
This patient’ s rare congenital heart defect presented an opportunity for a unique perfusion strategy during surgical repair. In nearly all other IAA repairs, the surgical team must place a second arterial cannula in the PDA to provide lower body circulation. In the absence of APW, most patients also have a VSD, which will allow for mixing of oxygenated blood to the right side of the heart. In this case, however, there was an intact ventricular septum, and the APW provided mixing of blood from the left to the right side. As such, the APW was a direct conduit to the descending aorta through the PDA, allowing for the utilization of a single arterial cannula.
Despite having a rare congenital heart defect, patients diagnosed with IAA and APW often have a good prognosis and a high chance of survival with proper surgical intervention [ 3 ]. Without intervention, this cardiac malformation has significant morbidity and mortality. Both double-patch( employed here) and single-patch techniques have been shown to have good clinical outcomes with no difference in the success of the procedure. Single-stage repair is more common than staged repair for this defect [ 2 ]. Few patients require reoperations, and overall mortality remains low throughout childhood. The most common re-intervention for this defect is for aortic arch obstructions. Pulmonary stenosis may also occur, so the child should be followed as they grow and develop to ensure their great arteries remain competent [ 7 ]. For this patient, a singlestage repair with a double-patch technique was performed, which was necessary due to the limited amount of native tissue available for anastomosis and anatomical distance at the interruption between the ascending and descending aortas. Despite having a late diagnosis, our patient recovered quite well, showed no signs of permanent damage, and was discharged without concern.