Recurrent stroke from paradoxical embolism in a case with hereditary hemorrhagic telangiectasia
Keywords:
Rendu Osler Weber, hereditary hemorragic telangiectasia, pulmonary arteriovenous malformation, recurrent ischemic stroke, TCD bubble test, right to left shuntAbstract
Rendu Osler Weber or Hereditary Hemorrhagic Telangiectesia (HHT) is an autosomal dominant genetic disorder causing abnormal development of vasculature characterized by telangiectases and arteriovenous malformations of specific organs. The most common presentation of HHT is epistaxis; however ischemic stroke occasionally occurs due to paradoxical shunt such as pulmonary ateriovenous malformation (PAVM). We reported a 37-year-old woman presenting with recurrent ischemic stroke as a result of paradoxical emboli from PAVMs. The patient was brought to emergency department and the examination revealed left facial palsy and left hemiplegia. The CT angiography demonstrated complete occlusion of the distal right M1 MCA. Transcranial Doppler showed positive microbubble examination with suspected extracardiac shunts corresponding with positive bubble test from transesophgeal echocardiogram. Pulmonary angiography demonstrated 3 PAVMs and the shunts were successfully embolized. The patient was disabling due to large volume of the right MCA infarction
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