A Fatal Case of Postpartum Cerebral Angiopathy with Concurrent Cardiomyopathy

Abstract

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare postpartum angiopathic syndrome which can cause ischemic or hemorrhagic stroke. Peripartum cardiomyopathy (PPCM) is a non-ischemiccardiomyopathy that also occurs postpartum. Here we report a case with concurrent of RCVS and PPCM. 32 years old female presented with worsening headache since her vaginal delivery six days ago. For a sudden onset left hemiplegia CT head showed a large right basal ganglia intracerebral hemorrhage causing midline shift leading to craniotomy.Cerebral angiogram done the next day showed diffuse intracranial vasoconstriction suggestive of RCVS and this persisted on angiogram a week later. Initial echocardiogram was normal with an ejection fraction (EF) of 6065% that reduced to 15–20% a week later. Postpartum day 10 she had fixed, dilated pupils. CT brain showed tonsillar herniation with apnea test confirming brain death. This case is the first one to show the unique combination of two conditions, PPCM and RCVSand sheds more light into perhaps common pathophysiologic mechanisms. Key words: Postpartum Cerebral Angiopathy, Reversible Cerebral Vasoconstriction Syndrome, Peripartum cardiomyopathy, Subarachnoid hemorrhage.