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Published on: July 21, 2013
Reversible cerebral vasoconstriction syndrome: Is it more than just cerebral vasoconstriction?
Seby John1, Rula A Hajj-Ali2, David Min3
1Cerebrovascular Center, Cleveland Clinic, OH, USA.
Insights
Systemic vascular alterations are newly observed in reversible cerebral vasoconstriction syndrome (RCVS). Some RCVS patients experience cardiac dysfunction, with a few showing complete recovery from cardiomyopathy.
Area of Science:
- Cardiology
- Neurology
- Vascular Medicine
Background:
- Reversible cerebral vasoconstriction syndrome (RCVS) is typically characterized by cerebral artery spasms.
- Systemic vascular involvement beyond the brain has not been previously described in RCVS.
- Cardiac dysfunction has not been a recognized feature of RCVS.
Observation:
- This case series investigated cardiac function in RCVS patients during acute ictus.
- Transthoracic echocardiography (TTE) was used to assess left ventricular ejection fraction (LVEF) and wall motion abnormalities (WMA).
- 18 of 68 RCVS patients (26%) underwent TTE during ictus.
Findings:
- Three patients (17%) exhibited WMA on initial TTE.
- These patients were female, with no prior history of coronary artery disease or heart failure.
- WMA resolved in two patients; persistent global left ventricular dysfunction was noted in one patient at 90 days.
Implications:
- Cardiac ventricular abnormalities may represent a broader spectrum of RCVS manifestations.
- The observed cardiomyopathy might stem from localized coronary vasoconstriction leading to myocardial ischemia.
- RCVS-associated vasoconstriction may extend beyond cerebral vasculature to affect extracerebral organs.
Background:
Systemic vascular alterations have not been described in reversible cerebral vasoconstriction syndrome (RCVS). We present a case series of RCVS patients having cardiac dysfunction during ictus, with a subset showing complete resolution of cardiomyopathy.
Methods:
Retrospective case-series: Cardiac left ventricular ejection fraction (LVEF) and wall motion abnormalities (WMA) visualized on transthoracic echocardiography (TTE), performed during RCVS ictus and follow-up was analyzed.
Results:
Of 68 patients, 18 (26%) had a TTE performed around ictus. Three of 18 (17%) patients demonstrated WMA on initial TTE. All three patients were female without previous coronary artery disease or heart failure, and were asymptomatic from the cardiac dysfunction. WMA resolved completely on follow-up in Patients 1 and 2. Global LV dysfunction persisted for at least 90 days in Patient 3.
Conclusion:
Although the exact pathophysiology of the cardiomyopathy is uncertain, it may be related to localized coronary vasoconstriction causing myocardial ischemia/infarction. Vasoconstriction may not be limited to the cerebral vasculature and may involve extracerebral organs. Cardiac ventricular abnormalities may be a part of the RCVS spectrum.
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