Sudden cardiac death in young children with neurofibromatosis type 1

Kanter, Ronald J.; Graham, Matthew; Fairbrother, David; Smith, S. Victor

doi:10.1016/j.jpeds.2006.07.046

« Previous Next »The Journal of Pediatrics
Volume 149, Issue 5 , Pages 718-720, November 2006

Sudden cardiac death in young children with neurofibromatosis type 1

Division of Pediatric Cardiology, Department of Pediatrics, Duke University School of Medicine, Durham, NC; Division of Anatomic Pathology, Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, NC; and Division of Pediatric Cardiology, Department of Pediatrics, East Carolina University Brody School of Medicine, Greenville, NC.

Received 15 March 2006; received in revised form 7 June 2006; accepted 28 July 2006.

Patients with neurofibromatosis type 1 (NF1) can manifest a characteristic vasculopathy that in adults is rarely associated with fatal coronary artery occlusion. We describe the clinical and pathological findings from 2 unrelated young children with NF1, a similar vasculopathy affecting their coronary arteries, and sudden cardiac death.

Abbreviations: LAD, Left anterior descending coronary artery, LCx, Left circumflex coronary artery, LMCA, Left main coronary artery, MI, Myocardial infarction, NF1, Neurofibromatosis type 1, VF, Ventricular fibrillation