Reoperative Aortic Root, Ascending, and Arch Replacement with Damus-Kaye-Stansel Reconstruction after Fontan Palliation

P0282 

Case Video Submission 

Alexander Nissen (1), Bradley Leshnower (2), Mani Daneshmand (2), Joshua Rosenblum (3)

(1) Emory University, Atlanta, GA, (2) Emory University Hospital, Atlanta, GA, (3) Children's Healthcare of Atlanta, Atlanta, GA

Alexander Nissen    -  Contact Me
Emory University

*Bradley Leshnower    -  Contact Me
Emory University Hospital

*Mani Daneshmand    -  Contact Me
Emory University Hospital

Joshua Rosenblum    -  Contact Me
Children's Healthcare of Atlanta

Alexander Nissen    -  Contact Me
Emory University

Objective: We present a case requiring neoaortic root, ascending, and arch replacement with Damus-Kaye-Stansel (DKS) reconstruction for massive neoaortic dilatation and severe neoaortic insufficiency after previous single ventricle palliation in the setting of depressed systemic ventricular function.

Case Video Summary: Our patient is a 20-year-old male with history hypoplastic left heart syndrome, who underwent 3-stage palliation, culminating in a fenestrated lateral tunnel Fontan. His cardiac MRI demonstrated rapid aortic enlargement, now 8.7cm, with severe neo-AI, and moderately reduced systemic ventricular function with a RVEF of 33%. He was initially referred for transplant evaluation, but given his functional status and moderate sensitization, he would likely spend several years of further decline on the waitlist. After consideration he was offered high-risk reoperative neoaortic root, ascending and arch replacement with DKS reconstruction to both prevent aortic complication, as well as eliminate neo-AI, and attendant further decline in systemic ventricular function. Reoperative sternotomy was performed using standard techniques. The innominate artery and left common carotid artery were mobilized for later snaring during periods of circulatory arrest with sACP. The DKS was identified, freed with a rim of healthy tissue, and cardioplegia administered with prompt arrest. The aneurysmal arch was resected to isolate the head vessels and remaining proximal descending aorta, to which we anastomosed a beveled 28mm graft. After mobilization and neoaortic valve excision, we secured a bioprosthetic valved conduit. After aneurysm excision and relief of previous distortion, it was clear that the graft-to-graft anastomosis would not be able to be completed with room for an aortic clamp, instead requiring another period of circulatory arrest to re-establish aortic continuity. A site on the Valsalva portion of the root graft was then chosen for reattachment of the DKS. He recovered well, has seen in follow-up, and is doing well without functional limitations, and stable mild-moderate systemic ventricular dysfunction.

Conclusions: Our case highlights the successful management of a complex neoaortic root and ascending aneurysm with severe neoaortic insufficiency after single ventricle palliation, treated with replacement and Damus-Kaye-Stansel reconstruction.

Aortic Root