A report is presented of a 31 year female patient with Ehlers-Danlos syndrome type IV who presented a bilateral renal artery dissection and a hematoma of the ascending aorta simultaneously. She had had an uneventful delivery six months before. We also discovered a situs solitus with dextrocardia in this patient. These complications were managed conservatively. Unfortunately the patient died of a pulmonary infection and a multi-organ failure syndrome several days after her admission.
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