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Neurovascular complications of marfan syndrome: a retrospective, hospital-based study
Robert J Wityk1, Carla Zanferrari, Stephen Oppenheimer
1Department of Neurology, Johns Hopkins Hospital, Baltimore, Md, USA. rwityk@jhmi.edu
Background And Purpose:
Small case series have associated Marfan syndrome with cerebral and spinal ischemia or hemorrhage. However, there has been no investigation of the frequency and etiology of neurovascular disorders in a large series of Marfan patients.
Methods:
We conducted a retrospective, hospital-based study of all Marfan syndrome patients seen in an 8-year period. Records were reviewed in detail, and clinical characteristics of those with and without a neurovascular diagnosis compared.
Results:
Of 513 patients, 18 (3.5%) had a neurovascular diagnosis, as follows: transient ischemic attack (11), cerebral infarction (2), spinal cord infarction (2), subdural hematoma (2), and spinal subarachnoid hemorrhage (1). A cardioembolic source was identified in 12 of 13 patients with cerebral ischemia, as follows: prosthetic heart valves (9), mitral valve prolapse (2), and atrial fibrillation (1). Chronic anticoagulant therapy was a likely cause in 2 of 3 patients with hemorrhagic events. Compared with other Marfan syndrome patients, those with neurovascular events were older (39.6 versus 31.7 years, P=0.04) and more likely to be in atrial fibrillation (22.2% versus 3.2%, P=<0.01), to have prosthetic heart valves (61.1% versus 7.7%, P=0.001), and to be taking anticoagulant therapy (72.2% versus 16.1%, P<0.001). Aortic disease, a putative factor in the etiology of neurovascular complications, was present in equal measure in Marfan patients with and without neurovascular complications (78% versus 65%, P=NS).
Conclusions:
Neurovascular complications of Marfan syndrome are rare during 8 years of follow-up, and generally are ischemic in nature. A high-risk cardiac source was identified in the majority. A significant association with vascular dissection was not established.
Insights
Neurovascular complications in Marfan syndrome are rare, primarily ischemic, and linked to cardiac issues like prosthetic valves. This study investigated their frequency and causes in a large Marfan patient cohort.
Area of Science:
- Cardiovascular Medicine
- Neurology
- Genetics
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue.
- Previous studies suggest a link between Marfan syndrome and neurovascular events.
- The frequency and causes of these events in Marfan syndrome remain understudied.
Purpose of the Study:
- To determine the frequency of neurovascular disorders in a large Marfan syndrome patient cohort.
- To investigate the underlying causes and risk factors for neurovascular complications in Marfan syndrome.
Main Methods:
- Retrospective study of 513 Marfan syndrome patients over an 8-year period.
- Detailed review of patient records to identify neurovascular diagnoses.
- Comparison of clinical characteristics between patients with and without neurovascular events.
Main Results:
- 18 out of 513 (3.5%) Marfan patients experienced neurovascular events.
- Cerebral ischemia (TIA, infarction) was most common, often linked to cardioembolic sources (prosthetic valves, MVP, AF).
- Hemorrhagic events were associated with anticoagulant therapy; older age and specific cardiac conditions were risk factors.
Conclusions:
- Neurovascular complications are infrequent in Marfan syndrome, predominantly ischemic.
- High-risk cardiac sources, particularly prosthetic heart valves, are frequently identified.
- No significant association was found between neurovascular complications and vascular dissection.