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Cardiac Manifestations in a Western Moyamoya Disease Population: A Single Center Descriptive Study and Review

Neurosurgery, 2020-12, Vol.67 (Supplement_1) [Peer Reviewed Journal]

Copyright © 2020 by the Congress of Neurological Surgeons 2020 ;Copyright © 2020 by the Congress of Neurological Surgeons ;ISSN: 0148-396X ;EISSN: 1524-4040 ;DOI: 10.1093/neuros/nyaa447_304

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  • Title:
    Cardiac Manifestations in a Western Moyamoya Disease Population: A Single Center Descriptive Study and Review
  • Author: Larson, Tony S ; Savastano, Luis E ; Klaas, James ; Lanzino, Giuseppe
  • Subjects: Congenital diseases ; Neurosurgery ; Surgical techniques
  • Is Part Of: Neurosurgery, 2020-12, Vol.67 (Supplement_1)
  • Description: Abstract INTRODUCTION An embryological association between Moyamoya disease (MMD) and cardiac manifestations has been proposed. Data up to this point remains anecdotal, and the prevalence of cardiac manifestations in a Western MMD population is uncertain. METHODS Medical records of MMD patients who presented to our institution between 1990–2019 were retrospectively reviewed for the presence of various congenital cardiac malformations and concomitant CAD. The prevalence of congenital cardiac defects and CAD was determined. A literature search for prior cases of MMD with concomitant cardiac manifestations was performed. RESULTS A total of 181 MMD patients were included in our analysis, 139 (76.8%) of whom were Caucasian. Ten patients had cardiac manifestations (5.5%). There were six total MMD patients with congenital cardiac defects (3.3%). All patients with congenital defects were diagnosed in childhood. The prevalence of congenital defects in MMD was slightly higher than the general population as reported previously (0.8-1.2%). Four MMD patients had CAD (2.2%). The average age of patients with CAD was 41.0 years (SD = 12.3, range = 33–59) in our series and 33.1 years (SD = 15.0) in a review of prior reports. CONCLUSION Our findings support an association between MMD and cardiac manifestations. Further investigation is warranted in order to further characterize this potential relationship and shed light on a possible cardio-cephalic neural crest syndrome.
  • Publisher: Oxford: Oxford University Press
  • Language: English
  • Identifier: ISSN: 0148-396X
    EISSN: 1524-4040
    DOI: 10.1093/neuros/nyaa447_304
  • Source: ProQuest Central
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