Minimally invasive surgical aortic valve replacement in setting of pseudoxanthoma elasticum

We present a rare case of surgical aortic valve replacement (SAVR) in a patient with pseudoxanthoma elasticum (PXE). Our patient developed severe symptomatic aortic stenosis (AS) at a relatively young age of 61 despite a trileaflet valve. Notable intraoperative challenges included difficulties with arterial access and hemodynamic monitoring accuracy due to PXE effects on peripheral arteries. Additionally, the diseased valve itself was unusual in its very crumbly nature with micro-popcorn-like calcifications, which easily broke apart with handling. Lastly, rivaroxaban was chosen and warfarin avoided to anticoagulate for postoperative atrial fibrillation in PXE to prevent thromboembolism.