Aortic rupture in Erdheim's medial necrosis

History and clinical findings: A 75-year-old man was admitted with increasi ng dyspnoea and recurrent left-sided chest pain, at first during exercise a nd later at rest. No cardiovascular risk factors could be found. His past m edical history revealed mastectomy and radiotherapy for breast cancer and a n operation for benign prostate hyperplasia. At admission the patient was i n very poor conditions with marked orthopnoea. Bilateral moist rales were h eard over both lungs with a 3/6 diastolic murmur an cardiac auscultation. Investigations: Anterolateral ST segment depression in the ECC and signs of pulmonary oedema in chest X-ray were also noted. Echocardiography discover ed global reduced left ventricular contractility with aortic insufficiency (11 degrees) in mild aortic valve sclerosis. Coronary angiography demonstra ted marked dilatation of the coronary arteries without stenosis. The ascend ing aorta was dilated without angiographic signs of a dissection. Diagnosis, treatment and course: After medical treatment and a short period without symptoms the patient had to be resuscitated and died after a inten se attack of dyspnoea and chest pain. The autopsy revealed a focal dissecti on of the ascending aorta with a small aortic rupture caused by idiopathic Erdheim's medial-necrosis. Conclusion: Erdheim's medical necrosis is an important cause of aortic diss ection and aortic rupture. If symptoms of acute severe chest pain are prese nt and a coronary syndrome can be excluded, possible disease of the aorta s hould be investigated. The reported case demonstrates the short time window between onset of symptoms and the necessary treatment.