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We experienced a case of acute multiple organ ischemia and multiple organ failure due to atypical aortic coarctation (AAC). Since the patient's hemodynamics were too unstable to perform surgical revascularization, we performed urgent endovascular therapy (EVT) with a stent. Eventually, the patient achieved remission from multiple organ failure and a satisfactory clinical outcome. We feel that EVT for AAC is a sufficiently effective treatment option if the purpose of EVT is to save a patient's life in the acute phase. In the present case, spontaneous retroperitoneal bleeding (SRB) occurred after EVT of AAC, but this is a rare incident, although noteworthy in the clinical course.

A 52-year-old man with recurrent epistaxis and palpebral conjunctival telangiectasia visited our hospital for a follow-up checkup for gastrointestinal polyposis. At 48 years of age, he underwent Y-graft replacement for an abdominal aortic aneurysm. Arteriovenous malformation was detected in his lungs, and a genetic test revealed an SMAD4 mutation. Eventually, he was diagnosed with juvenile polyposis-hereditary hemorrhagic telangiectasia (JP-HHT) syndrome. In addition, fatty degeneration of the left ventricle and a coronary aneurysm were detected. This is the first report suggesting the possibility of an association between these manifestations and JP-HHT due to SMAD4 mutations. Examining cardiovascular disorders in JP-HHT patients is imperative.