Thursday, April 25, 2024: 5:38 PM - 7:00 PM
Sheraton Times Square
Room: Central Park
Objective: Aortic arch pathology in patients with congenital heart disease and prior aortic arch interventions can be complex. Repairs are technically demanding and require high-risk intraprocedural adjustments. Here, we present the case of a 15-year-old girl born with hypoplastic left heart syndrome, who had undergone four previous open-heart surgeries for single ventricle palliation, most recently an intra/extracardiac fenestrated Fontan procedure. The patient was found to have Fontan baffle narrowing along with extensive coral-reef-like reactive calcifications of the bovine jugular vein graft used for reconstruction of the ascending aorta and aortic arch. The patient underwent successful arch repair and Damus-Kay-Stansel (DKS) revision.
Methods: Given the Fontan baffle narrowing, along with significant arch obstruction from calcification as demonstrated by chest CT (Figure 1A), a stepwise procedural plan was developed including stenting of the Fontan conduit prior to arch replacement. Virtual and printed 3D models of the aorta were utilized to assist with surgical planning (Figure 1B). Right femoral vein cannulation was performed under fluoroscopy guidance and the right axillary artery was exposed for bypass cannulation. A fifth median sternotomy was undertaken, and cardiopulmonary bypass was initiated via femoral vein and axillary artery. The entire mediastinal dissection was performed with aortic no-touch technique to minimize risk of embolization. A period of antegrade cerebral perfusion was started by clamping the distal innominate artery. The left carotid and left subclavian arteries were clamped. The ascending aorta was transected at the level of the DKS, and myocardial arrest was achieved via direct ostial administration of cardioplegia. The ascending aorta was near-totally obstructed, with only a minimal (3-4mm) residual lumen (Figure 1C). The ascending aorta and base of the arch were resected en bloc with the calcified matter (Figure 1D). The arch was reconstructed with a Gelweave graft, and the DKS was re-anastomosed.
Results: Intraoperative transesophageal echocardiogram demonstrated a patent arch and DKS. No neo-aortic valve regurgitation was observed. The procedure was performed successfully with avoidance of any neurologic event. Her post-operative course was complicated by a hoarse voice and video swallow study revealing aspiration with thin liquids. She was last seen in outpatient clinic on post-operative day 50 continuing her work with speech therapy and progressing appropriately.
Conclusions: Aortic pathology in patients with congenital heart disease and prior arch interventions represent a complex surgical problem and requires careful preoperative planning. A hybrid approach with preoperative catheter intervention for optimization and minimization of surgical repair is recommended when feasible. This approach can mitigate the risk for perioperative complications and increase procedural success.
Authors
Cathlyn Medina (1), Berk Aykut (2), Lauren Parker (3), Lindsey Reynolds (4), Stephen Miller (4), Joseph Cao (4), Joseph Turek (5), Douglas Overbey (6), Ziv Beckerman (3)
Institutions
(1) N/A, N/A, (2) Duke Univ Med Ctr, Dept. of Surgery, N/A, (3) Duke University Medical Center, Durham, NC, (4) Duke University, Durham, NC, (5) Duke Children's Hospital, Durham, NC, (6) Duke University, United States
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