Coagulation system activated in Duchenne muscular dystrophy patients with cardiac dysfunction

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Published on: April 10, 2019

Toshio Saito1, Yuko Yamamoto, Tsuyoshi Matsumura

  • 1Division of Neurology, National Hospital Organization Toneyama National Hospital, 5-1-1 Toneyama, Toyonaka, Osaka 560-8552, Japan. saitot@toneyama.hosp.go.jp

Brain & Development
|August 27, 2005
PubMed

Insights

Activated coagulation is linked to cardiac dysfunction in Duchenne muscular dystrophy (DMD). Significantly elevated thrombin-antithrombin complex (TAT) and prothrombin fragment (F1+2) were found in DMD patients with severely depressed left ventricular ejection fraction (LVEF).

Area of Science:

  • Cardiovascular Medicine
  • Hematology
  • Neuromuscular Disorders

Background:

  • Duchenne muscular dystrophy (DMD) is a progressive genetic disorder primarily affecting skeletal muscles.
  • Cardiac dysfunction is a common and serious complication in DMD patients.
  • The relationship between coagulation abnormalities and cardiac dysfunction in DMD requires further investigation.

Purpose of the Study:

  • To investigate basic abnormalities of coagulation and fibrinolysis in Duchenne muscular dystrophy patients with varying degrees of cardiac dysfunction.
  • To determine if specific coagulation markers correlate with left ventricular ejection fraction (LVEF) in DMD patients.

Main Methods:

  • Forty-seven DMD patients (aged 13-37 years) were enrolled and categorized into three groups based on LVEF determined by echocardiography: markedly depressed (<30%), slightly depressed (30-50%), and normal (>50%).
  • Serum levels of total fibrin and fibrinogen degradation products (FDP) were measured.
  • Plasma levels of fibrinogen, thrombin-antithrombin complex (TAT), prothrombin fragment (F1+2), and D-dimer were quantified.

Main Results:

  • Significantly elevated levels of thrombin-antithrombin complex (TAT) and prothrombin fragment (F1+2) were observed in the markedly depressed LVEF group compared to the other groups.
  • No significant differences in serum FDP, plasma fibrinogen, or D-dimer levels were found among the three LVEF groups.
  • These findings suggest a specific activation of the coagulation system in DMD patients with severe cardiac impairment.

Conclusions:

  • Activated coagulation, indicated by elevated TAT and F1+2, is associated with cardiac dysfunction in Duchenne muscular dystrophy patients.
  • These specific markers may serve as indicators of heightened thrombotic risk or underlying procoagulant state in DMD with cardiac involvement.
  • Further research is warranted to explore the clinical implications and potential therapeutic targets related to coagulation activation in DMD.

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