Marfan syndrome and cardiovascular complications: results of a family investigation

BMC Cardiovasc Disord. 2017 Jul 19;17(1):193. doi: 10.1186/s12872-017-0629-8.

Abstract

Background: Cardiovascular complications in Marfan syndrome (MFS) make all its seriousness. Taking as a basis the Ghent criteria, we conducted a family screening from an index case. The objective was to describe the clinical characteristics of MFS anomalies and to detect cardiovascular complications in our patients.

Case presentation: Six subjects were evaluated. Patients had to be in the same uterine siblings of the index case or be a descendant. The objective was to search for MFS based on the diagnostic criteria of Ghent and, subsequently, detecting cardiovascular damage. The average age was 24 years. The examination revealed three cases of sudden death in a context of chest pain. Five subjects had systemic involvement with a score ≥ 7 that allowed to the diagnosis of MFS. Two patients had simultaneously ectopia lentis and myopia. In terms of cardiovascular damage, there were three cases of dilatation of the aortic root, two cases of aortic dissection of Stanford's type A with severe aortic regurgitation in one case and moderate in the other. There were three patients with moderate mitral regurgitation with a case by valve prolapse.

Conclusion: The family screening is crucial in Marfan syndrome. It revealed serious cardiovascular complications including sudden death and aortic dissection.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aortic Aneurysm / diagnosis
  • Aortic Aneurysm / etiology*
  • Aortic Aneurysm / genetics
  • Aortic Dissection / diagnosis
  • Aortic Dissection / etiology*
  • Aortic Dissection / genetics
  • Aortic Valve Insufficiency / diagnosis
  • Aortic Valve Insufficiency / etiology*
  • Aortic Valve Insufficiency / genetics
  • Aortography / methods
  • Computed Tomography Angiography
  • Death, Sudden, Cardiac / etiology*
  • Echocardiography
  • Fatal Outcome
  • Female
  • Genetic Predisposition to Disease
  • Genetic Testing
  • Heredity
  • Humans
  • Male
  • Marfan Syndrome / complications*
  • Marfan Syndrome / diagnosis
  • Marfan Syndrome / genetics
  • Mitral Valve Insufficiency / diagnosis
  • Mitral Valve Insufficiency / etiology*
  • Mitral Valve Insufficiency / genetics
  • Pedigree
  • Phenotype
  • Prognosis
  • Risk Factors
  • Young Adult