The unravelling of primary myocardial impairment in Marfan syndrome by modern echocardiography

Heart. 2009 Oct;95(19):1561-6. doi: 10.1136/hrt.2008.152934. Epub 2009 Feb 17.

Abstract

Use of echocardiography has dramatically changed the way in which patients with Marfan syndrome are diagnosed, monitored and treated. Owing to the lethal nature of aortic complications, priority has been given to the assessment of the aortic root. Echocardiographic studies on patients with Marfan syndrome have also provided data supporting primary myocardial involvement, although this evidence has remained controversial for several years. Use of more sensitive ultrasound techniques has demonstrated mild myocardial impairment in these patients. Biventricular function assessment should be added to the aortic root evaluation, so that appropriate treatment may be offered to support myocardial function.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Arrhythmias, Cardiac / diagnostic imaging
  • Arrhythmias, Cardiac / epidemiology
  • Arrhythmias, Cardiac / etiology
  • Arrhythmias, Cardiac / therapy
  • Cardiomyopathies / diagnostic imaging*
  • Cardiomyopathies / etiology
  • Child
  • Echocardiography / methods*
  • Humans
  • Marfan Syndrome / complications
  • Marfan Syndrome / diagnostic imaging*
  • Ventricular Dysfunction, Left / epidemiology
  • Ventricular Dysfunction, Left / therapy
  • Ventricular Dysfunction, Right / epidemiology
  • Ventricular Dysfunction, Right / therapy