A case report recently presented at the American College of Cardiology Annual Scientific Session describes a patient with aggressive systemic mastocytosis (SM) who had a history of failed transcatheter aortic valve replacement (TAVR).
TAVR is a minimally invasive procedure that is used to replace damaged aortic valves. Patients with aggressive SM can experience significant organ damage due to elevated mast cell activation. Cardiovascular complications, including valve dysfunction and heart failure, may occur in some individuals.
The case report described a 67-year-old woman who underwent TAVR in 2016. After the first valve failed, she needed a subsequent replacement procedure, known as valve-in-valve TAVR, in 2020.
The patient had a prior medical history of aggressive SM and lymphoma. She presented with heart failure in 2022, leading the physicians to suspect potential involvement of SM in the repeated failures of these artificial valves.
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After treating the patient for heart failure, the authors decided to pursue a third valve replacement procedure called valve-in-valve-in-valve TAVR (ViViV-TAVR). Implantation of a mechanical valve via open surgery was also considered. However, due to to the patient’s risk status, including an increased risk of bleeding, this was ultimately decided against.
The third valve replacement was well-tolerated with no post-operative complications. The patient was prescribed midostaurin to reduce mast cell proliferation, with the goal of preventing degeneration of the newly implanted valve.
In 2024, an echocardiography was performed, which revealed that the valve was still functioning as expected.
“We believe this to be the first reported case of TAVR failure associated with [aggressive] SM treated with ViViV-TAVR,” said the report’s authors. “Although [aggressive] SM is rare, clinicians must be aware of potential complications, particularly early valve failure and atherosclerosis.”
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