Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome

J Pediatr. 2007 Jan;150(1):77-82. doi: 10.1016/j.jpeds.2006.09.003.

Abstract

Objective: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients.

Study design: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied. All clinical data and 213 serial echocardiograms were reviewed, and aortic root dimensions were measured. Patients were divided into 2 groups for comparison: untreated (n = 34) and treated (n = 29).

Results: At study entry, the 2 study groups were comparable in terms of age, sex, body surface area (BSA), aortic root measurements, heart rate, and corresponding z scores. Follow-up duration in each group was similar. At last follow-up, heart rates and heart rate z scores were lower in the treated group. Rates of change of aortic root measurements (P = .52) and the corresponding z scores were not statistically different between the 2 group at the study's end.

Conclusions: This study suggests that that beta-blocker therapy does not significantly alter the rate of aortic root dilation in children with Marfan syndrome. Based on these data, the recommendation of lifetime beta-blocker therapy instituted during childhood should be reassessed.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adrenergic beta-Antagonists / therapeutic use*
  • Aorta, Thoracic / diagnostic imaging*
  • Aorta, Thoracic / drug effects
  • Atenolol / therapeutic use
  • Child
  • Dilatation, Pathologic / diagnostic imaging
  • Dilatation, Pathologic / etiology
  • Disease Progression
  • Echocardiography
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Male
  • Marfan Syndrome / complications
  • Marfan Syndrome / diagnostic imaging
  • Marfan Syndrome / prevention & control*
  • Retrospective Studies
  • Time Factors
  • Treatment Outcome

Substances

  • Adrenergic beta-Antagonists
  • Atenolol