Biomedical

Reversible cerebral vasoconstriction syndrome post-cardiac transplantation: a therapeutic dilemma: case report







  Peer Reviewed

Abstract

Key Questions


1. What is reversible cerebral vasoconstriction syndrome (RCVS)?

RCVS is characterized by diffuse, multifocal segmental narrowing of cerebral arteries that can result in ischemic stroke. It is often caused by immunosuppressant pharmacotherapy.

2. What are the common causes of RCVS in post-transplant patients?

RCVS is typically induced by immunotherapy drugs such as steroids and calcineurin inhibitors (e.g., tacrolimus, cyclosporine).

3. How is RCVS diagnosed?

Diagnosis is based on clinical symptoms such as severe headaches and transient neurological deficits, alongside imaging studies like cerebral angiography showing multifocal vasoconstriction.

4. What are the treatment options for RCVS?

Treatment focuses on removing the triggering agent (e.g., adjusting immunotherapy), along with supportive care like calcium channel blockers (e.g., nimodipine) to relieve vasospasm.


Abstract


Background

Background Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by diffuse, multifocal segmental narrowing of cerebral arteries and can result in ischaemic stroke. Causal factors, identified in 60% of cases, include immunosuppressant pharmacotherapy. The few reports following heart transplantation are almost all in Asian recipients. We report on a Caucasian Australian patient with immunotherapy induced RCVS post heart transplantation to highlight the state of knowledge of the condition and the treatment dilemma it poses.

Case presentation

Case presentation A 51-year-old female underwent orthotopic heart transplantation at our institution. Induction immunotherapy comprised basiliximab, mycophenolate mofetil and methylprednisolone. On day 6 post-transplantation the patient was transitioned to oral prednisolone and tacrolimus. On day 7 the patient began to experience bilateral, severe, transient occipital and temporal headaches. On day 9 tacrolimus dose was up-titrated. A non-contrast computed tomography brain (CTB) was normal. Endomyocardial biopsy on day 12 demonstrated moderate Acute Cellular Rejection (ACR), which was treated with intravenous methylprednisolone. That evening the patient experienced a 15-minute episode of expressive dysphasia. The following morning she became confused, aphasic, and demonstrated right sided neglect and right hemianopia. A CT cerebral perfusion scan demonstrated hypoperfusion in the left middle cerebral artery (MCA) territory and cerebral angiography revealed widespread, focal multi-segmental narrowing of the anterior and posterior circulations. A diagnosis of RCVS was made, and nimodipine was commenced. As both steroids and tacrolimus are potential triggers of RCVS, cyclosporin replaced tacrolimus and methylprednisolone dose was reduced. A further CTB demonstrated a large left MCA territory infarct with left M2 MCA occlusion. The patient made steady neurological improvement. She was discharged 34 days post-transplantation with mild residual right lower limb weakness and persistent visual field defect on verapamil, cyclosporine, everolimus, mycophenolate mofetil and prednisolone.

Conclusion

Conclusion Reversible cerebral vasoconstriction syndrome is rare after orthotopic heart transplantation. Until now, RCVS has been almost exclusively described in Asian recipients, and is typically caused by immunotherapy. The condition may lead to permanent neurological deficits, and in the absence of definitive treatments, early recognition and imaging based diagnosis is essential to provide the opportunity to remove the causal agent(s). Co-existent ACR, can pose unique treatment difficulties.