[Aortic rupture in idiopathic Gsell-Erdheim medial necrosis]

Abstract

History and clinical findings: A 75-year-old man was admitted with increasing dyspnoea and recurrent left-sided chest pain, at first during exercise and later at rest. No cardiovascular risk factors could be found. His past medical history revealed mastectomy and radiotherapy for breast cancer and an operation for benign prostate hyperplasia. At admission the patient was in very poor conditions with marked orthopnoea. Bilateral moist rales were heard over both lungs with a 3/6 diastolic murmur an cardiac auscultation.

Investigations: Anterolateral ST segment depression in the ECG and signs of pulmonary oedema in chest X-ray were also noted. Echocardiography discovered global reduced left ventricular contractility with aortic insufficiency (II degree) in mild aortic valve sclerosis. Coronary angiography demonstrated marked dilatation of the coronary arteries without stenosis. The ascending 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 intense attack of dyspnoea and chest pain. The autopsy revealed a focal dissection 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 dissection and aortic rupture. If symptoms of acute severe chest pain are present and a coronary syndrome can be excluded, possible disease of the aorta should be investigated. The reported case demonstrates the short time window between onset of symptoms and the necessary treatment.