Abstract

Introduction: Postpartum Cerebral Angiopathy (PCA) or Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare but important postpartum syndrome characterized by severe headaches with complications including seizures and intracranial hemorrhages. Peripartum cardiomyopathy (PPCM) is a known non-ischemic cardiomyopathy occurring mostly in the immediate postpartum period. This case is one of its kind to show the unique combination of PPCM and PCA. Case Report: 32 years old Hispanic female with no past medical history presented with worsening headache since epidural anesthesia used for her uneventful spontaneous vaginal delivery six days ago. While in ED she suddenly developed left hemiplegia and an immediate CT head showed right basal ganglia intracerebral hemorrhage causing 10mm right to left midline shift. An emergent frontotemporal craniotomy and evacuation of hemorrhage was done. A cerebral angiogram the next day showed diffuse cerebral arterial vasoconstriction responding to intra-arterial verapamil suggestive of RCVS. An echocardiogram was completely normal and vasculitis workup was negative. Patient was started on nimodipine however she became hypotensive requiring pressors. A week later repeat angiography showed persistent vasospasm and a repeat echocardiogram showed dilated left and right ventricles with global impairment in systolic function and an EF of 15-20%. The subsequent day there was a change in her neurological status where she lost all brain and brainstem reflexes, pupils got fixed and dilated. CT brain showed global edema and tonsillar herniation. Apnea test was done which was consistent with the diagnosis of brain death. Discussion: The pathophysiologic mechanism of RCVS is believed to be related to altered cerebral auto regulation in response to endothelial injury that usually resolves in 3 months. The diagnosis can be made by cerebral angiography, MRA or CTA that shows multifocal segmental arterial constrictive lesions. It is likely that a combination of diffuse persistent cerebral vasospasm accompanied by poor cardiac function that culminated in eventual brain death of this healthy, postpartum patient. Management includes calcium channel blockers, sequential cerebral angiograms and supportive care.

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