Increased Prevalence of Cerebrovascular Disease in Hospitalized Patients with Marfan Syndrome

Sarasa T. Kim, Harry Cloft, Kelly Flemming, David F Kallmes, Giuseppe Lanzino, Waleed Brinjikji

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


Background and Purpose: Small studies have suggested that Marfan syndrome is associated with a number of cerebrovascular complications. We sought to determine whether a clinical diagnosis of Marfan syndrome is associated with a higher prevalence of cerebrovascular diseases than the general population by performing a case-control study of hospitalized patients in the Nationwide Inpatient Sample (NIS). Methods: Using the 2000-2012 NIS, we performed a case-control study matching cases of Marfan syndrome to controls without such a diagnosis. The prevalence of various cerebrovascular diseases between the 2 groups were compared, and multivariate logistic regression was used to adjust for suspected comorbidities. Results: Between 2000 and 2012, there were a total of 13,883 discharges carrying a diagnosis of Marfan syndrome. On univariate analysis, patients with Marfan syndrome were more likely to have a primary or secondary diagnosis of hemorrhagic stroke (0.5% versus 0.3%, odds ratio [OR] = 1.56, 95% confidence interval [CI] = 1.06-2.29, P = 0.02) as well as intracranial hemorrhage (subarachnoid hemorrhage [SAH] and hemorrhagic stroke) (0.3% versus 0.2%, OR = 1.72, 95% CI = 1.05-2.82, P = 0.03). Patients hospitalized with Marfan syndrome were significantly more likely to have carotid dissection (0.3% versus 0.0%, OR = 11.69, 95% CI = 3.60-38.08, P <. 0001) and cerebral aneurysms (0.2% versus 0.1%, OR = 3.67, 95% CI = 1.76-7.68, P = 0.0002). On multivariate analysis adjusted for age, race, and comorbidities, patients with Marfan syndrome had significantly higher odds of ischemic stroke (OR = 1.20, 95% CI = 1.02-1.43, P = 0.03), hemorrhagic stroke (OR = 1.75, 95% CI = 1.18-2.63, P = 0.005), carotid artery dissection (OR = 11.94, 95% CI = 4.23-50.03, P < 0.0001), and cerebral aneurysm (OR = 3.95, 95% CI = 1.95-8.90, P <. 0.0001). Conclusions: There is a modestly increased prevalence of ischemic stroke, hemorrhagic stroke, and cerebral aneurysms in hospitalized patients with Marfan syndrome when compared with controls.

Original languageEnglish (US)
JournalJournal of Stroke and Cerebrovascular Diseases
StateAccepted/In press - 2017


  • Carotid aneurysm
  • Carotid dissection
  • Cerebral aneurysm
  • Cerebrovascular malformation
  • Hemorrhagic stroke
  • Ischemic stroke
  • Marfan syndrome
  • Vertebral dissection

ASJC Scopus subject areas

  • Surgery
  • Rehabilitation
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine


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