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Pseudoxanthoma elasticum (PXE) is a rare hereditary disorder that causes elastic tissue degeneration in the skin, eyes, and cardiovascular system. Gastrointestinal bleeding and fundus hemorrhage are serious complications associated with PXE prognosis as well as cardiovascular involvement. This is a rare case of acute coronary syndrome in a PXE patient with high bleeding risk. LEARNING OBJECTIVE: Pseudoxanthoma elasticum (PXE) resulting in acute coronary syndrome (ACS) is rare. Given PXE patients are generally at very high bleeding risk, antithrombotic therapy as secondary prevention after ACS onset should be taken into full consideration.

A 39-year-old woman developed a pulmonary embolism at 28 weeks of gestation, after a 4-week period of bedrest, and required emergencycesarean section due to a decrease in fetal heart rate. Pulseless electrical activity (PEA) developed after intravenous anesthesia. The fetus was delivered 5 min after PEA onset, during cardiopulmonary resuscitation of the mother. Intravenous recombinant tissue-plasminogen activator injection, percutaneous cardiopulmonary support, and 24-h hypothermia therapy were administered to the mother, followed by inferior vena cava filter insertion, combined with catheter thrombus fragmentation and percutaneous thrombectomy. Both the patient and her baby survived.

A 67-year-old man was admitted to our hospital due to chest pain at rest. Seven years previously, the patient underwent percutaneous coronary intervention (PCI) of the left ascending artery and implanted sirolimus-eluting stent (SES). Coronary angioscopy (CAS) performed at that time showed a white plaque at the SES site. Two years after the first PCI, repeat CAS demonstrated light yellow plaques at the SES site. At the time of his presentation to our hospital, coronary angiography showed in-stent restenosis at the SES site, and CAS demonstrated the plaque rupture with presence of dense yellow plaque and various thrombi. After distal protection, drug-eluting balloon treatment was performed. Collected specimens from culprit sites included foamy macrophages, cholesterin crystals, neutrophils, and fibrin, suggesting that progressive neoatherosclerosis at the SES site triggered the acute coronary syndrome. This study highlights the importance of ensuring careful patient follow-up after SES implantation. .