Abstract
Hemolytic anemia caused by kinking of a prosthetic graft after total arch replacement (TAR) is rare. While many reported cases involve a single angulated ("L-shaped") kink, clinically significant hemolysis is not always observed. We report a unique case of a 68-year-old man who developed progressive aortic regurgitation (AR) and subsequent severe hemolytic anemia ten years after TAR with an elephant trunk using a Triplex® graft for acute type A aortic dissection. Early postoperative imaging showed a mild, single curvature of the ascending graft without evidence of hemolysis. Over time, the graft progressively deformed into a distinct S-shaped kink, accompanied by worsening AR and eventual onset of severe hemolysis. Laboratory findings confirmed hemolysis, and surgical intervention with a Bentall procedure and redo TAR led to prompt resolution of symptoms and laboratory abnormalities. This case highlights that not all graft kinking result in hemolysis, but the evolution to a double-kink (S-shaped) configuration may critically alter hemodynamics, particularly when compounded by progressive AR. Awareness of this progression and careful morphological surveillance of the graft are essential for early detection and timely management. To our knowledge, this is the first report documenting the evolution from a single to S-shaped graft kink as a key trigger for late-onset hemolysis.